Download A practical guide to understanding cancer

A practical guide to
understanding cancer
Contents
Contents
About this booklet
4
What is cancer?
5
The lymphatic system
7
Soft tissue sarcomas
8
Types of soft tissue sarcoma
10
Risk factors and causes
15
Symptoms
18
How soft tissue sarcomas are diagnosed
19
Further tests 23
Grading and staging
27
Treatment overview
31
Surgery
36
Limb-sparing surgery
40
Amputation
44
Radiotherapy
50
Chemotherapy
57
Targeted therapies
65
After treatment – follow-up
67
Research – clinical trials
70
Your feelings
73
What you can do
77
1
Understanding soft tissue sarcomas
Who can help?
78
If you are a relative or friend
79
Relationships
81
Talking to children
82
Financial help and benefits 83
Work
85
How we can help you
87
Other useful organisations
91
Further resources 97
Your notes and questions
101
TNM staging
102
2
Understanding soft tissue sarcomas
About this booklet
This booklet is about a type of cancer called
soft tissue sarcoma. We hope it answers some
of your questions and helps you deal with some
of the feelings you may have.
We can’t advise you about the best treatment for you.
This information can only come from your own doctor,
who knows your full medical history.
If you’d like to discuss this information, call the Macmillan
Support Line free on 0808 808 00 00, Monday–Friday,
9am–8pm. If you’re hard of hearing you can use textphone
0808 808 0121, or Text Relay. For non-English speakers,
interpreters are available. Alternatively, visit macmillan.org.uk
Turn to pages 91–100 for some useful addresses and websites,
and page 101 to write down any notes or questions for your
doctor or nurse.
If you find this booklet helpful, you could pass it on to your
family and friends. They may also want information to help
them support you.
4
What is cancer?
What is cancer?
The organs and tissues of the body are made up of tiny
building blocks called cells. Cancer is a disease of these cells.
Cancer isn’t a single disease with a single cause and a single
type of treatment. There are more than 200 different kinds of
cancer, each with its own name and treatment.
Although cells in different parts of the body may look
different and work in different ways, most repair and
reproduce themselves in the same way. Normally, cells divide
in an orderly and controlled way. But if for some reason the
process gets out of control, the cells carry on dividing, and
develop into a lump called a tumour. Tumours can be either
benign (non-cancerous) or malignant (cancerous). Doctors
can tell whether a tumour is benign or malignant by removing
a piece of tissue (biopsy) and examining a small sample of
cells under a microscope.
Normal cells
Cells forming a tumour
5
Understanding soft tissue sarcomas
In a benign tumour, the cells do not spread to other parts of
the body and so are not cancerous. However, they may carry
on growing at the original site, and may cause a problem by
pressing on surrounding organs.
In a malignant tumour, the cancer cells have the ability to
spread beyond the original area of the body. If the tumour
is left untreated, it may spread into surrounding tissue.
Sometimes, cells break away from the original (primary)
cancer. They may spread to other organs in the body through
the bloodstream or lymphatic system (see next page).
When the cancer cells reach a new area, they may go
on dividing and form a new tumour. This is known as a
secondary cancer or a metastasis.
6
The lymphatic system
The lymphatic system
The lymphatic system is part of the immune system – the body’s
natural defence against infection and disease. It’s made up
of organs such as bone marrow, the thymus, the spleen, and
lymph nodes. The lymph nodes throughout the body are
connected by a network of tiny lymphatic tubes (ducts).
The lymphatic system has two main roles: it helps to protect
the body from infection and it drains fluid from the tissues.
Neck (cervical)
lymph nodes
Thymus
Armpit
(axillary)
lymph nodes
Diaphragm
Spleen
Groin
(inguinal)
lymph nodes
The lymphatic system
7
Understanding soft tissue sarcomas
Soft tissue sarcomas
Sarcomas are rare. About 2,300 people a year in the UK are
diagnosed with a sarcoma. This includes bone sarcomas as
well as soft tissue sarcomas.
Soft tissue sarcomas are cancers that develop from cells in
the soft, supporting tissues of the body. They can occur in soft
tissues such as fat, muscle, nerves, fibrous tissues, blood vessels
or in any of the other tissues that support, surround and protect
the organs of the body.
Soft tissue sarcomas can develop in any part of the body.
Most of them develop in the arms and legs, but they can also
develop in specific organs such as the womb (uterus), stomach,
skin and small bowel. Less commonly, they may occur in the
head and neck.
Some types of sarcoma occur in children, teenagers and young
adults, but generally they are more likely to develop in people
over the age of 30.
Bone sarcomas
Some sarcomas, such as osteosarcoma, start in the bone.
These grow and develop differently and are treated differently
from soft tissue sarcomas. Occasionally, it’s hard to tell whether
a sarcoma has started in soft tissue or bone. There are some
types of sarcoma, such as Ewing’s tumours (see page 14),
that can begin in either the bone or soft tissue.
We can send you a separate booklet about cancer that
starts in the bone.
8
Soft tissue sarcomas
9
Understanding soft tissue sarcomas
Types of soft tissue sarcoma
There are many types of soft tissue sarcoma, and they tend
to grow and develop differently. The most common types are
described in this section. Your doctor or specialist nurse can tell
you more about which type you have.
Each type of sarcoma is named after the type of cell it started
from, rather than the part of the body where it started to grow.
Fat tissue tumours
Sarcomas that start in the body’s fat cells are called
liposarcomas. They can grow anywhere in the body and
most commonly affect people aged 50–65. Some grow very
slowly, taking many years to develop, whereas others grow
more quickly.
Muscle tissue tumours
There are two different types of muscle tissue in the body:
smooth and skeletal.
Smooth muscle sarcomas
Smooth muscle forms the walls of internal organs such as the
womb (uterus), stomach, intestine and blood vessels. The muscle
causes these organs to contract, which happens without our
control. Smooth muscle is also called involuntary muscle.
Sarcomas that develop in smooth muscle are called
leiomyosarcomas. They are one of the more common
types of sarcoma and can occur anywhere in the body,
especially in the back of the abdominal area (retroperitoneum).
10
Types of soft tissue sarcoma
Leiomyosarcomas are less often found in the deep, soft tissues
of the legs or arms. They tend to occur in adults, particularly in
the elderly.
Skeletal muscle sarcomas
Skeletal muscles are the active muscles in our arms and legs
or other parts of the body that we control. They are voluntary
muscles and sometimes called striated muscles because the
cells look stripy when examined under a microscope.
Sarcomas that grow in the voluntary muscles of the body are
called rhabdomyosarcomas. They occur mostly in the head
and neck, but also in organs such as the bladder, vagina and
the arms or legs. Rhabdomyosarcomas are more commonly
diagnosed in children than in adults.
Peripheral nerve tumours
The nervous system in the body is divided into two parts:
the central nervous system, which is made up of the brain and
spinal cord; and the peripheral nervous system, which consists
of all the nerves that run throughout the body.
Sarcomas of the peripheral nerves develop in the cells that
cover the nerves. They’re known as malignant peripheral
nerve sheath tumours (MPNST) and can occur anywhere
in the body. There are different types of MPNSTs, including
malignant schwannomas and neurofibrosarcomas. They most
commonly occur in people who have a rare genetic disorder
called neurofibromatosis (von Recklinghausen’s disease).
11
Understanding soft tissue sarcomas
Fibrous tissue tumours
Fibrous tissues join together the inner structures of the body,
for example, they join muscles to bones. This tissue is made
up of cells called fibrocytes. A sarcoma of the fibrous tissue is
called a fibrosarcoma. They are most commonly found on the
arms, legs or trunk, but can occur deeper in the body. They can
occur at any age but are more commonly seen in people aged
20–60. Most people first notice them as a painless, firm lump.
Myxofibrosarcomas
This is a type of fibrosarcoma. It was previously called
malignant fibrous histiocytoma (MFH). It’s not clear which type
of cell it starts from. Myxofibrosarcomas can affect any part of
the body, but most commonly occur in the arms or legs.
Fibromatosis
These tumours are sometimes known as Desmoid tumours.
They are classified as benign tumours, not cancer. They’re
sometimes said to be halfway between a fibrosarcoma and
a benign fibroma (a non-cancerous tumour of fibrous tissue).
Desmoid tumours don’t tend to spread to other parts of the body,
but can spread into nearby tissues, and so are usually treated
in a similar way to cancerous sarcomas.
Joint tissue tumours
Soft tissue sarcomas that develop very close to the body’s joints
are known as synovial sarcomas. They commonly develop
near, but not inside, joints such as the knee or elbow, but they
can occur in any part of the body. They usually appear as hard
lumps and are more common in children and young adults.
12
Types of soft tissue sarcoma
Blood and lymph vessel tumours
Sarcomas that start from the cells that make up the walls
of blood or lymph vessels are called angiosarcomas.
Haemangiosarcomas develop from blood vessels and
lymphangiosarcomas develop from the lymph vessels.
Angiosarcomas sometimes occur in a part of the body that has
been treated with radiotherapy many years before.
Other types of sarcoma
Gastrointestinal stromal tumours (GIST)
These are the most common type of soft tissue sarcoma
and develop in nerve cells in the walls of the digestive system.
The digestive system is often called the gastrointestinal (GI) tract.
This type of tumour may also be called GI stromal sarcoma.
GISTs behave differently from other types of sarcoma and are
treated very differently.
Kaposi’s sarcoma (KS)
Although Kaposi’s sarcoma is a type of sarcoma, it differs from
other sarcomas in the way it develops. It starts from cells in
the skin. Coloured patches or lumps can develop in the skin,
mouth, lymph nodes or internal organs such as the lung, liver
or spleen.
Kaposi’s sarcoma can affect people with a weakened immune
system, including people with HIV and Aids. Other types
can affect people of Jewish, Italian and West African origin.
Kaposi’s sarcoma is treated differently from other types of soft
tissue sarcoma.
13
Understanding soft tissue sarcomas
Ewing’s tumours
Ewing’s tumours are a type of bone sarcoma, but about a third
of all Ewing’s tumours develop in the soft tissue and are known
as extraosseous Ewing’s tumours. Soft tissue Ewing’s sarcomas
tend to behave differently from other soft tissue sarcomas and
are usually treated in a similar way to bone sarcomas.
We have further information about all the types of soft
tissue sarcoma mentioned in this section.
Rarer types of sarcoma
There are other much rarer types of sarcoma than those we
have already listed. These include:
•• alveolar soft part sarcoma
•• dermatofibrosarcoma protuberans (DFSP)
•• desmoplastic small round cell tumour
•• epithelioid sarcoma
•• extraskeletal myxoid chondrosarcoma
•• giant cell fibroblastoma (GCF).
Our cancer support specialists on 0808 808 00 00
can give you information about these rarer types
of sarcoma.
14
Risk factors and causes
Soft tissue sarcomas in children
Soft tissue sarcomas can occur in children, particularly some
types of rhabdomyosarcoma. Their symptoms and treatment
may differ from those in an adult with the same sarcoma.
We can send you a booklet called A parent’s guide
to children’s cancer, which discusses the treatment
of children’s cancers. We also have cancer information
sheets about some types of sarcoma that occur
in children.
Risk factors and causes
Although the causes of soft tissue sarcomas are unknown,
research is going on to try to find out more. Sarcomas,
like other cancers, are not infectious and can’t be passed
on to other people.
Age
Sarcomas can occur at any age but are more common as
people get older. About two-thirds of all soft tissue sarcomas
are diagnosed in people over 50.
Genetic conditions
Most sarcomas are not caused by an inherited faulty gene that
can be passed on to other family members. Members of your
family are not likely to have an increased risk of developing a
soft tissue sarcoma because you have one.
15
Understanding soft tissue sarcomas
However, people who have some rare inherited genetic
conditions are more at risk of developing a sarcoma.
These conditions include neurofibromatosis, Gardner’s
syndrome, Li-Fraumeni syndrome and retinoblastoma.
You would normally know if any member of your family
had one of these conditions, and their doctor would check
them regularly for any sign of a sarcoma.
Previous radiotherapy treatment
Very rarely, a soft tissue sarcoma will occur in a part of the
body that has previously been treated with radiotherapy for
another type of cancer. The sarcoma will not usually develop
until at least 5–10 years after the radiotherapy treatment.
To reduce the risk, radiotherapy is very carefully planned.
Improvements in the way radiotherapy is given mean that
the risk of developing a sarcoma is very small.
Exposure to chemicals
The development of some sarcomas may be linked to
exposure to some types of chemicals. The chemicals include:
vinyl chloride, which is used for making plastics; some types of
herbicides (weedkillers); and dioxins, which are a waste product
produced during the manufacture of chemicals and fertilisers.
Injury
There is no evidence that an injury can cause a soft tissue
sarcoma to develop. It’s possible that an injury may draw
attention to a sarcoma that was already there and not causing
any symptoms, but the sarcoma will have taken many years
to develop.
16
Understanding soft tissue sarcomas
Symptoms
Sarcomas often don’t cause any symptoms for a long time.
They can start in any part of the body and the symptoms will
depend on the part of the body that’s affected.
If the sarcoma is in an arm or a leg, the most common
symptom is an uncomfortable swelling in the affected limb.
Occasionally, this swelling may be painful or tender, but it
may also be painless.
If the sarcoma is in the central part of the body (the trunk),
the symptoms will depend on which of the body’s organs is
affected. For example:
•• A sarcoma in a lung may cause a cough and breathlessness.
•• A sarcoma in the abdomen could cause abdominal pain,
vomiting and constipation. A GIST (see page 13) may also
cause bleeding into the bowel. This may cause you to vomit
blood, pass dark bowel motions or develop symptoms of
anaemia (a low number of red blood cells), such as shortness
of breath or tiredness.
•• A sarcoma affecting the womb may cause bleeding from
the vagina and pain in the lower part of the abdomen.
Usually, soft tissue sarcomas don’t cause any symptoms until
they are quite large and pressing on an organ in the body or
on a nerve or muscle. Things to look out for include:
•• any lump, especially if it is increasing in size and is bigger
than 5cm (2in)
•• any lump that is painful or tender
18
How soft tissue sarcomas are diagnosed
•• any lump that is deep in the body (ie not just under the skin)
•• any lump that has come back after being surgically removed.
If you notice any of the above, contact your GP,
but remember that these symptoms can also be
caused by other conditions.
How soft tissue sarcomas
are diagnosed
You’ll usually begin by seeing your GP, who will ask you
about your symptoms and examine you. They may arrange
for you to have tests such as an ultrasound of the lump.
You may need to be referred to hospital for these tests and
for specialist advice and treatment. If your GP suspects that
you might have a sarcoma, you’ll usually be seen at the
hospital within two weeks.
At the hospital
The specialist will ask about your general health and any
previous medical problems. They will also examine you,
which will include feeling the area where there is pain or
swelling. You may be asked to have blood tests and a chest
x-ray to check your general health.
19
Understanding soft tissue sarcomas
Biopsy
The doctors will usually ask if they can take a sample of the
lump, which will be examined under a microscope. This is
known as a biopsy and is the only way to tell whether the lump
is a cancerous or non-cancerous (benign) tumour. You may
have other tests and scans (see pages 23–26) to assess the
lump before a biopsy. A biopsy can be done in two ways:
a core needle biopsy or a surgical biopsy.
Core needle biopsy
This is when a sample of cells is removed from the lump using
a needle. Several samples may be taken.
Before the biopsy is taken, a local anaesthetic is injected to
numb the area. If the lump is near the surface of your body
and can easily be felt, the doctor will probably just feel it
to guide the needle in. If the lump is deep within the body
(such as in the abdomen) or is harder to feel, the doctor
will use an ultrasound scan or sometimes a CT scan
(see pages 24–25) to guide the needle into the right place.
A pathologist (a doctor who specialises in diagnosing disease
by looking at body tissue and cells) will look at the cells under
a microscope to see whether they are benign or cancerous.
If the lump is a sarcoma, further tests may be done on the
sample to try to find out exactly what type of sarcoma it is.
Sometimes, particularly with children, the biopsy is done under
a general anaesthetic while you are asleep.
For most people, a core needle biopsy will show whether the
lump is a sarcoma or not. However, sometimes not enough
20
How soft tissue sarcomas are diagnosed
cells are collected to give a clear answer, and then a surgical
biopsy is needed.
Surgical biopsy
This is far less commonly used. It will only be done if a core
needle biopsy doesn’t give a definite result. A surgical knife
(scalpel) is used to open the area and remove a tissue
sample from the lump. If the lump is small enough, all of
it may be removed.
A surgical biopsy may be done under a local or general
anaesthetic, depending on the position of the lump and
how deep it is within the body. As with a core needle biopsy,
the sample will be sent to the laboratory so that it can be
tested. Often a large number of studies will be done even
on a very small sample.
It can take up to ten days to get all the results. This can be a
worrying time, but it’s very important that an accurate diagnosis
is made so that the most appropriate treatment can be given.
If the lump turns out to be benign, you may not need to have
any more treatment. If it’s cancer, your doctor will discuss the
treatment options with you.
Our cancer support specialists on 0808 808 00 00
can offer you support during this time.
21
Further tests
Further tests
If the tests show that you have a soft tissue sarcoma, you may
need further tests to see exactly where the cancer is and whether
it has spread. The results of all these tests help your doctors
gather as much information as possible, so they can decide
what the best treatment is for you.
Other tests may include the following:
Chest x-ray
This will be done to check your general health and to look
for any sign that the cancer has spread to your lungs, as this
is one of the most common places for soft tissue sarcomas to
spread to.
MRI (magnetic resonance imaging) scan
This test uses magnetism to build up a detailed picture of areas
of your body. The scanner is a powerful magnet so you may
be asked to complete and sign a checklist to make sure it’s
safe for you. The checklist asks about any metal implants you
may have, for example a pacemaker, surgical clips or bone
pins. You should also tell your doctor if you’ve ever worked with
metal or in the metal industry as very tiny fragments of metal
can sometimes lodge in the body. If you do have any metal
in your body, it’s likely that you won’t be able to have an MRI
scan. In this situation another type of scan can be used.
Before the scan, you’ll be asked to remove any metal
belongings, including jewellery. Some people are given an
injection of dye into a vein in the arm, which doesn’t usually
23
Understanding soft tissue sarcomas
cause discomfort. This is called a contrast medium and can
help the images from the scan to show up more clearly.
During the test you’ll lie very still on a couch inside a long
cylinder (tube) for about 30 minutes. It’s painless but can
be slightly uncomfortable, and some people feel a bit
claustrophobic. It’s also noisy, but you’ll be given earplugs
or headphones. You can hear, and speak to, the person
operating the scanner.
CT (computerised tomography) scan
A CT scan takes a series of x-rays, which build up a
three-dimensional picture of the inside of the body.
The scan takes 10–30 minutes and is painless.
Someone having a CT scan
24
Further tests
It uses a small amount of radiation, which is very unlikely to
harm you and will not harm anyone you come into contact
with. You will be asked not to eat or drink for at least four
hours before the scan.
You may be given a drink or injection of a dye, which allows
particular areas to be seen more clearly. This may make you feel
hot all over for a few minutes. It’s important to let your doctor
know if you are allergic to iodine or have asthma, because you
could have a more serious reaction to the injection.
You’ll probably be able to go home as soon as the scan is over.
Ultrasound scan of the abdomen
This may be used if you have an abdominal lump.
An ultrasound scan uses sound waves to produce pictures
of internal organs such as the liver and the inside of the
abdomen. You’ll usually be asked not to eat or drink for
a few hours before the test.
Once you’re lying comfortably on your back, a gel is spread
on to your abdomen. A small device like a microphone is
passed over the area. This produces sound waves, which are
then converted into a picture by a computer. The test only
takes a few minutes.
If the lump is in your womb, the ultrasound scan may be done
by inserting an ultrasound device into your vagina. This is known
as a transvaginal ultrasound scan and gives a very clear picture
of the womb.
25
Understanding soft tissue sarcomas
PET (positron emission tomography) scan
PET scans can be used to accurately define the cancer and
find out if it has spread to other parts of the body. PET scans
can also be used to examine any lumps that remain after
treatment to see if they are scar tissue or whether cancer cells
are still present.
A PET scan uses low-dose radioactive glucose (a type of sugar)
to measure the activity of cells in different parts of the body.
It may help to find out if a tumour is growing and whether it is
cancerous (malignant) or non-cancerous (benign).
A very small amount of a mildly radioactive substance is
injected into a vein, usually in your arm. A scan is then taken a
couple of hours later. Cancer cells are usually more active than
surrounding tissue, and show up on the scan. Not all hospitals
have PET scanners, so if you need one you may have to travel
to another hospital.
Waiting for test results
It may take up to two weeks for the results of your tests to
be ready, and a follow-up appointment will be made for you.
This waiting period can often be a very anxious time and it
may help you to talk things over with a relative, close friend,
your specialist nurse at the hospital or one of the support
organisations on pages 91–97.
You can also call 0808 808 00 00 and speak to one of our
cancer support specialists.
26
Grading and staging
Grading and staging
The information from your biopsy, scans and other tests will tell
your doctors more about the grade and stage of your cancer.
This information will be used by a team of doctors and nurses
known as the multidisciplinary team or MDT (see page 31),
who will decide on the most appropriate treatment for you.
Grading
The grade of a cancer gives an idea of how quickly it might
grow. Doctors examine the cancer cells under a microscope
and see how they compare with normal cells. The grade helps
your doctor decide if you need further treatment after surgery. •• Grade 1 or low-grade or well differentiated The cancer
cells look similar to normal cells and usually grow slowly and
are less likely to spread.
•• Grade 2 or moderate- or intermediate-grade The cancer
cells look more abnormal and are slightly faster-growing.
•• Grade 3 or high-grade or poorly differentiated
The cancer cells look very different from normal cells and
may grow more quickly.
Grading of soft tissue sarcomas can sometimes be difficult,
especially for the less common types. Your doctor will be able
to talk to you in more detail about grading.
27
Understanding soft tissue sarcomas
Staging
The stage of a cancer describes its size and whether it has
spread beyond its original area in the body.
Several different staging systems may be used for soft tissue
sarcomas. Two of the most commonly used systems are a
number staging system and the TNM staging system.
Number staging
In this system, there are four main stages from 1–4, which may
be further subdivided:
Stage 1A The cancer is small (5cm or less) and either
low-grade or the grade isn’t known. It can either be close to the
surface of the body (superficial) or deep within the body.
Stage 1B The cancer is larger than 5cm and either low-grade
or the grade isn’t known. It can be either superficial or deep
within the body.
Stage 1 soft tissue sarcomas have not spread to lymph
nodes or other parts of the body.
Stage 2A The cancer is small (5cm or less) and either
moderate- or high-grade. It can either be superficial or
deep within the body.
Stage 2B The cancer is larger than 5cm and moderate-grade.
It can be either superficial or deep within the body.
Stage 2 soft tissue sarcomas have not spread to lymph
nodes or other parts of the body.
28
Grading and staging
Stage 3 The cancer is bigger than 5cm and high-grade.
It can be either superficial or deep within the body, and has
not begun to spread. OR The cancer is of any size, superficial
or deep and of any grade, but it has spread to at least one
lymph node, but not to other parts of the body.
Stage 4 The cancer can be any size, superficial or deep,
and of any grade. It may or may not have spread to lymph
nodes, but it has spread to another part of the body such
as the lungs, liver, bones or soft tissues. This is known as
secondary or metastatic cancer.
The TNM staging system is more complicated than
the number staging system. We have more detailed
information about TNM staging at the back of this
booklet if you’d like to know more – see pages 102–104.
29
Treatment overview
Treatment overview
As sarcomas are rare cancers, you should always be referred
for treatment at a specialist sarcoma unit where a team of
specialist doctors and others work together. This is known as
a multidisciplinary team (MDT) and will include:
•• a surgeon who specialises in sarcomas
•• an oncologist who specialises in cancer treatments such as
chemotherapy, radiotherapy and biological therapy
•• a pathologist who specialises in diagnosing disease by
looking at body tissue and cells
•• a radiologist who analyses x-rays and scans
•• a specialist nurse who gives information and support.
The multidisciplinary team may also include other healthcare
professionals such as a dietitian, physiotherapist, therapy
radiographer, occupational therapist and a psychologist
or counsellor.
In order to plan the best treatment for you, your doctors will
take into account your general health, and the type, stage and
grade of the sarcoma.
Surgery is the most common treatment (see pages 36–49).
If the soft tissue sarcoma is small and it’s possible to remove
it completely, surgery may be used on its own.
For larger sarcomas, and where there may be a possibility of
cancer cells being left behind, radiotherapy is usually used
as well as surgery. Radiotherapy may be given before the
31
Understanding soft tissue sarcomas
operation to shrink the tumour and make it easier to remove.
It may also be used afterwards to try to destroy any cancer cells
that may not have been removed (see pages 50–56).
Sometimes, radiotherapy is used on its own or in combination
with chemotherapy to treat more advanced sarcomas that
can’t be removed.
Chemotherapy (see pages 57–64) may sometimes be given
before surgery to shrink the tumour. For some types of sarcoma
it may also be given after surgery to try to destroy any cancer
cells that have not been removed. Chemotherapy may also be
used if the sarcoma has already spread or comes back after
initial treatment.
For gastrointestinal stromal tumours a targeted therapy
called imatinib (Glivec®) may be used before or after surgery
(see page 65).
Second opinion
Your multidisciplinary team (MDT) uses national treatment
guidelines to decide the most suitable treatment for you.
Even so, you may want another medical opinion. If you feel
it will be helpful, you can ask either your specialist or GP to
refer you to another specialist for a second opinion. Getting a
second opinion may delay the start of your treatment, so you
and your doctor need to be confident that it will give you useful
information. If you do go for a second opinion, it may be a
good idea to take a relative or friend with you, and have a list
of questions ready, so that you can make sure your concerns
are covered during the discussion.
32
Treatment overview
Giving your consent
Before you have any treatment, your doctor will explain its aims.
They will ask you to sign a form saying that you give permission
(consent) for the hospital staff to give you the treatment.
No medical treatment can be given without your consent,
and before you are asked to sign the form, you should be
given full information about:
•• the type and extent of the treatment
•• its advantages and disadvantages
•• any significant risks or side effects
•• any other treatments that may be available.
If you don’t understand what you’ve been told, let the
staff know straight away, so they can explain again.
Some cancer treatments are complex, so it’s not unusual
to need repeated explanations.
It’s a good idea to have a relative or friend with you when the
treatment is explained, to help you remember the discussion.
You may also find it useful to write a list of questions before
your appointment.
People sometimes feel that hospital staff are too busy to answer
their questions, but it’s important for you to know how the
treatment is likely to affect you. The staff should be willing to
make time for your questions.
You can always ask for more time if you feel that you can’t
make a decision when your treatment is first explained to you.
33
Understanding soft tissue sarcomas
You are also free to choose not to have the treatment. The staff
can explain what may happen if you don’t have it. It’s essential
to tell a doctor or the nurse in charge, so they can record your
decision in your medical notes. You don’t have to give a reason
for not wanting treatment, but it can help to let the staff know
your concerns so they can give you the best advice.
The advantages and disadvantages of treatment
Many people are frightened by the idea of having cancer
treatments, particularly because of the side effects that can
occur. However, these can usually be controlled with medicines.
Treatment can be given for different reasons and the potential
benefits will vary depending upon your individual situation.
Early-stage sarcoma
In people with early-stage sarcoma, surgery is often done with
the aim of curing the cancer, and additional treatments may
also be given to reduce the risk of it coming back.
Advanced-stage sarcoma (metastatic)
If the cancer is at a more advanced stage, the treatment
may only be able to control it, leading to an improvement
in symptoms and a better quality of life. However, for some
people, the treatment will have no effect on the cancer and
they will get the side effects of treatment with little benefit.
34
Treatment overview
Treatment decisions
If you have early-stage cancer and have been offered
treatment that aims to cure it, it may be easy to decide whether
to accept the treatment.
However, if a cure is not possible and the treatment is being
given to control the cancer for a period of time, it may be more
difficult to decide whether to go ahead. Making decisions about
treatment in these circumstances is always difficult, and you
may need to discuss in detail with your doctor whether you
wish to have treatment. If you choose not to, you can still be
given supportive (palliative) care, with medicines to control
any symptoms.
It’s important that you ask your doctors and nurses any
questions you have about your treatment. The more you
understand about your treatment, the easier it will be for you.
35
Understanding soft tissue sarcomas
Surgery
As soft tissue sarcomas are rare, surgery is usually carried out
by a surgeon who specialises in treating them. The surgeon
will often be part of a multidisciplinary team and be based
in a specialist bone (orthopaedic) hospital, or in a large
cancer hospital.
Sometimes, people have a lump removed and only find out it’s
a soft tissue sarcoma after the surgery. In this situation, you will
be referred to a sarcoma specialist who is part of an MDT after
your initial surgery.
The aim of most sarcoma surgery is to remove the tumour
completely without leaving anything behind. Usually, an
operation called a wide local excision is done. This means
removing the cancer along with a border (margin) of healthy,
cancer-free tissue all around it. The border of healthy tissue is
removed to reduce the risk of the cancer coming back in that
area. If the tumour is in the abdomen, it can sometimes be
difficult to remove it with enough normal tissue to get clear
margins. This is because the tumour may be very close to
vital organs.
It’s difficult to give general information about sarcoma surgery
because the type of operation you have will depend on where
in your body the sarcoma is. Many sarcomas are in the arm
or leg, but they can grow anywhere. Your surgeon will discuss
your operation with you in detail.
36
Surgery
Surgery to the chest or abdomen
Surgery is usually the main treatment for soft tissue sarcomas of
the trunk (the chest and abdomen). The extent of the operation
will depend on the exact position of the sarcoma in the body.
Your doctor will discuss this fully with you before the operation.
After your surgery, you will have a tube (drip) going into a vein
in your arm. This will give you fluids and nutrients for 2–3 days
until you can eat and drink properly again. The nurses on the
ward will then take the drip out. Your doctor will let you know
when you can start to eat again.
Radiotherapy may also be used after operations for sarcomas
in the chest or abdomen to try to make sure that any remaining
cancer cells are destroyed.
Surgery to the arms or legs
Over the past few years, major improvements have been
made in surgery for soft tissue sarcomas of the arms and legs.
In the past, an operation to remove the limb (amputation) was
usually needed if cancer was found. A false limb (prosthesis)
would then be made to replace the limb that had been
removed. Amputation is still sometimes needed, but it’s now
usually possible to remove just the cancer and some of the
tissue around it. This is known as limb-sparing surgery and
is done by using a combination of surgery, radiotherapy and
occasionally chemotherapy (see pages 40–43).
37
Understanding soft tissue sarcomas
Plastic surgery
Depending on the size and position of your tumour,
the surgeon may have to remove a large area of tissue.
So you may need to have some reconstructive/cosmetic
(plastic) surgery to repair the area. If this is needed, it will
be done at the same time as your operation.
The aim of plastic surgery is to make the area look and
function as naturally as possible. The exact surgery you have
will depend on where in your body the sarcoma is and how
much tissue the surgeon needs to remove. If only a small area
has been removed, the surgeon may be able to join the two
edges together again. But for larger areas, a skin graft may be
needed. This involves taking some skin from a different part
of the body (known as the donor site) and using it to repair the
operation site. Both sites will gradually heal over a few weeks
after the operation.
Occasionally, surgeons need to repair the operation site with
a specialist technique using skin and tissue. This is known as a
tissue flap and there are several different types. The surgeon
will explain your operation to you and answer any questions
you have.
Secondary tumours
Surgery is sometimes used to remove sarcoma that has spread
to other parts of the body. An operation is usually only possible
if the secondary tumours are small and there are only a few
of them. It’s mainly done when the sarcoma has spread to the
lungs, but may be considered for tumours in other parts of the
body. If this operation is necessary in your case, your doctor will
discuss it with you fully.
38
Understanding soft tissue sarcomas
Limb-sparing surgery
People who have a soft tissue sarcoma can sometimes have
limb-sparing surgery. This is when just the area of cancer,
and some of the tissue around it, is removed. This avoids the
need for amputation.
Before your operation, your doctor and the nurses on the ward
will discuss your treatment with you in detail. It often helps to
talk to someone who has had the same operation. The medical
staff should be able to arrange this for you. On some wards,
a clinical nurse specialist may be available to discuss any
worries that you have.
If you’ve had chemotherapy, your body will need time to
recover before limb-sparing surgery. Usually it takes at least
a couple of weeks after chemotherapy before you are ready
to have your operation.
Your doctor and anaesthetist will see you before the operation
to make sure you understand what is going to happen and
answer any questions you have. You will have to sign a consent
form agreeing to the surgery – this, too, is a good time to ask
any questions. Many people find that the more they know
about what is going to happen, the less frightening it seems.
If you have body hair in the area that will be operated on,
you will need to be shaved before your operation. This is
done to reduce the risk of infection. You may be shaved in
the operating theatre after you’ve had your anaesthetic
(when you’re asleep).
40
Limb-sparing surgery
After your operation
At first, your limb will be firmly bandaged. This is to give the
area time to heal. You will probably have one or two drainage
tubes in the wound to remove any fluid that collects in the
area of the operation. They will be removed once the fluid has
stopped draining, usually after a few days.
Swelling around the wound
Sometimes, fluid can build up around the wound, especially
following surgery to a buttock or limb. The swelling should
gradually reduce over a few weeks. Sometimes, a lot of fluid
builds up around the wound – this is known as a seroma.
It may need to be drained by a doctor or nurse.
Nutrition and fluids
You may have a drip (infusion) going into a vein in your arm.
This will give you fluids and nutrients for 2–3 days until you are
able to eat and drink properly again. The nurses on the ward
will then take the drip out.
Pain
Pain can usually be controlled effectively with painkillers.
You’ll need to take these for a few days after your operation.
They may be given into a vein (intravenously), into the
space around your spinal cord (epidural), into a muscle
(intramuscularly) or as tablets.
To start with, you will probably need a strong painkiller such as
morphine. You may be given intravenous pain relief through a
syringe connected to an electronic pump. The pump can be set
to give you a continuous dose of painkiller. You may also have
a handset with a button you can press if you feel sore. This is
called patient controlled analgesia (PCA). It’s designed so that
41
Understanding soft tissue sarcomas
you can’t have too much painkiller (an overdose), so it’s okay
to press it whenever you’re uncomfortable.
If you’ve had surgery to your leg, you may have pain relief
using an epidural. This is a fine tube that’s inserted through
your back into the area just outside the membranes around
your spinal cord, which is called the epidural space. A local
anaesthetic can be continuously given into this space to numb
the nerves that run to your legs.
Let your nurses and doctors know as soon as possible if you’re
in pain. This will help them give you the combination and dose
of painkillers that’s right for you.
Exercises
Your nurses or the physiotherapist will teach you breathing and
leg exercises. You can help yourself get better by doing the
exercises as often as you need to. Breathing exercises will help
to prevent you from getting a chest infection. Leg exercises will
help stop clots forming in your legs. Chest infections or blood
clots can develop if you’re not moving around as much as you
normally would be.
Your nurses will encourage you to get up and about as soon as
possible. However, limb-sparing surgery is a major operation,
and you may have to stay in bed for some time afterwards.
This may range from a couple of days to up to a week.
A physiotherapist will show you exercises to keep the muscles in
the limb strong and supple. This is so you can use it normally
once it’s strong enough. The physiotherapist will also help you
move the limb once you’re up and about again. Sometimes,
you will need a brace or support for your limb. If the operation
42
Limb-sparing surgery
was on your leg, you may be given crutches to use at first,
while you’re learning to walk again. You will probably be left
with a slight limp.
Sometimes, walking with a limp can put pressure on the leg
and cause pain elsewhere in the body. Although it’s important
to have any continuing pain checked by your doctor, it doesn’t
necessarily mean that the cancer has come back.
Going home
Most people are able to go home once their wound has
healed, usually about 7–10 days after their operation.
Radiotherapy
After limb-sparing surgery, radiotherapy treatment is usually
given to the area of the operation to destroy any cancer cells
that may still be in the area. This is done because it’s very
difficult to be completely certain that all the cancer cells were
removed during the operation.
We discuss radiotherapy on pages 50–56.
43
Understanding soft tissue sarcomas
Amputation
It’s not always possible to do limb-sparing surgery and
occasionally amputation may be necessary. This may
be because it’s the only way to get rid of the cancer.
Very occasionally, after discussion with their specialist
doctor and family, people choose to have an amputation
instead of limb-sparing surgery.
The preparation for amputation is similar to that for
limb-sparing surgery (see page 40). Psychological support
for people who are about to have an amputation is also
very important. The nursing and medical staff looking after
you will be able to offer help and support. It may also be
helpful to talk to someone who has had the same operation
and can give you practical advice as well as support.
After your operation
You will usually have a drip for a few days to give you fluids.
A bandage will be applied over the affected site to help shape
the area. You’ll have a tube in the wound to drain off any fluid
that builds up.
Immediately after your surgery, your pain will usually be
controlled either by an epidural, a patient controlled analgesia
(PCA) pump or painkillers by injection (see pages 41–42).
Some people have a pain that appears to come from the
part of the limb that has been amputated. This is known as
phantom pain or sensation. Although this pain will usually fade
over time, there may be some discomfort in the area for a while
after the operation.
44
Amputation
Our booklet Controlling cancer pain gives information
about pain and the many ways of controlling it.
About 2–3 days after surgery, you will be encouraged and
helped to move around. The physiotherapist will visit you shortly
after your operation and show you how to do exercises to keep
the muscles around the operation site strong and supple. This
will make it easier to use an artificial limb. The physiotherapist
will also show you how to do the breathing and leg exercises
described on pages 42–43.
Artificial limb (prosthesis)
Following an amputation, most people can be fitted with an
artificial limb called a prosthesis. Modern technology means
that artificial limbs are now very effective, enabling people
to walk, run and play sport.
The fitting of artificial limbs may be arranged through the ward
where you’re being treated or through a separate limb clinic.
After your operation, you’ll have an appointment to see an
artificial limb specialist who will show you the different types of
prosthesis and how they work. Careful measurements have to
be taken so that the prosthesis fits properly. It may take several
weeks for your prosthesis to be made and, in the meantime,
you may be fitted with a temporary one so that you can begin
to get used to having one. Your doctor or nurse can give you
more information about this.
After a couple of weeks and once your wound has healed,
you’ll be able to go home.
45
46
Amputation
Living with an amputation
Amputation is very distressing and it can take a long time
to come to terms with it.
Feelings and emotions
Losing an arm or a leg can feel like a bereavement. You will
need time to grieve for your loss and to start to cope with the
emotional and practical difficulties this type of surgery can
bring. Our booklet How are you feeling? discusses some of the
emotions and feelings that a diagnosis of cancer can cause, and
includes organisations available to help you cope with them.
Body image
Even if you thought you had a good idea of what to expect
before surgery, you may still feel shocked and distressed after
the operation, when the full realisation of having lost an arm or
leg hits you. You will be used to what your body looks like and
it can be very difficult to come to terms with a major change
such as an amputation.
Feeling like you look different from other people can affect
your self-confidence. You may feel afraid of being rejected –
both socially and sexually. You may, at times, even wish that
you’d never agreed to the operation.
At first, you may find it difficult to see people after your
amputation. But as you and the people close to you become
used to the way you look, you will become more confident
about dealing with the reactions of people you don’t know.
Some people find it helpful to get out and about as soon as
possible after their operation. However, it’s important to take
the time you need to get used to your amputation and to do
things in your own time. You may want to take someone with
47
Understanding soft tissue sarcomas
you at first to give you emotional support. You may find that
other people don’t even notice your amputation, especially if
you’re wearing an artificial limb.
You may find it useful to read our booklet Coping with
body changes after cancer.
Family and friends
Your partner, family and friends may also find it hard to come
to terms with their feelings about your amputation. You may be
anxious about what they will say or think, and whether you will
be able to cope with their reactions. This worry can feel very
real, but most people find that their families and friends want to
do as much as possible to support them. It can help to be open
about your feelings and any fear of rejection.
Our booklet, Talking about your cancer, can help you
find ways of talking to family and friends.
Sexuality
You may feel unattractive and embarrassed about your body,
and worry that no one will find you sexually attractive again.
Meeting a new partner may seem particularly daunting. If you
have a partner, you may be concerned that they will not find
you attractive any more.
We can send you a booklet called Sexuality and cancer,
which discusses these issues. We also have a booklet
for young people called Relationships, sex and fertility
for young people affected by cancer.
48
Amputation
Help is available
You will need time and help to come to terms with your
emotions, which are likely to be very strong. The hospital
staff will know this and will try to help you all they can.
Often, there are counsellors or psychologists in the hospital,
and the staff can arrange for you to see them.
Many people find it helpful to discuss their feelings in depth
with a close friend or someone who is removed from their
situation, such as a counsellor. Our cancer support specialists
on 0808 808 00 00 can give you information about
counsellors in your area.
49
Understanding soft tissue sarcomas
Radiotherapy
Radiotherapy treats cancer by using high-energy x-rays to
destroy cancer cells, while doing as little harm as possible
to normal cells.
Radiotherapy is an important part of treatment for soft tissue
sarcomas, particularly those affecting the arms or legs.
It’s usually given after surgery to destroy any remaining
cancer cells and to reduce the risk of the cancer coming
back. The wound must be healed before radiotherapy starts.
Sometimes, radiotherapy is given before an operation
(neo-adjuvant radiotherapy), either to shrink the tumour
and make it easier for the surgeon to remove it successfully,
or to avoid having to amputate the limb.
Radiotherapy is sometimes given on its own and may be the
only treatment needed. Radiotherapy is also given to relieve
symptoms and control sarcomas that can’t be removed.
Radiotherapy for sarcomas is normally given as a series of
short daily treatments in the hospital radiotherapy department.
High-energy x-rays are directed from a machine at the area of
the cancer.
The treatments are usually given from Monday–Friday,
with a rest at the weekend. The number of treatments will
depend on the type, size and position of the sarcoma. A course
of treatment for early cancer will usually last about six weeks.
Each treatment takes about 10–15 minutes. Your doctor will
discuss the treatment and possible side effects with you.
50
Radiotherapy
Radiotherapy for sarcomas is often given using a technique
called conformal radiotherapy. The radiation beams are
shaped to the part of the body that needs to be treated.
For some people, a more complex technique called
intensity-modulated radiation therapy (IMRT) may be
used. IMRT delivers a high dose of radiation to the tumour and
varies the dose given to nearby healthy tissue. This can help
to reduce side effects.
Planning your radiotherapy
To make sure that the radiotherapy is as effective as possible,
it has to be carefully planned. Planning ensures the radiotherapy
rays are aimed precisely at the cancer and cause the least
possible damage to the surrounding healthy tissues. The
treatment is planned by a specialist doctor known as a clinical
oncologist. Planning is important and may take a few visits.
If your sarcoma is in your arm or leg, you may need to have
a plastic mould made. The mould helps to keep the limb
still during radiotherapy treatment sessions. It’s made in the
mould room on your first visit to the radiotherapy department.
Your doctor or specialist nurse will tell you more about moulds
if one is needed.
As part of the planning, you’ll have a CT scan taken of the
area to be treated (see pages 24–25). At the same time,
therapy radiographers will take measurements from you,
which are needed for treatment planning. This session will
usually take about 30 minutes.
You may also need to have an MRI scan (see pages 23–24).
This uses powerful magnetic fields to give a detailed picture of
part of your body, which can give additional useful information.
51
Understanding soft tissue sarcomas
The radiographer’s measurements and the information from
the scans are fed into the radiotherapy planning computer to
help your doctors plan your treatment more precisely.
Marks may be drawn on your skin, or on the mould, to help
the radiographer (who gives you your treatment) to position
you accurately and to show where the rays are to be directed.
These marks must stay visible throughout your treatment,
but they can be washed off once your course of treatment is
over. Often, very small tattoo marks are also made on the skin.
These marks are permanent and will only be done with your
permission. It’s a little uncomfortable while the tattoo is being
made, but it’s a good way of making sure that treatment is
directed accurately.
At the beginning of your radiotherapy, you’ll be told how to
look after the area being treated.
Treatment sessions
At the beginning of each session of radiotherapy, the
radiographer will position you carefully on the couch and
make sure you’re comfortable. During your treatment you’ll
be alone in the room, but you can talk to the radiographer
who will watch you from the next room. Radiotherapy is not
painful, but you will have to lie still for a few minutes during
the treatment.
52
Radiotherapy
Someone being positioned for radiotherapy
Stereotactic radiotherapy
This is a new way of giving radiotherapy that may sometimes
be used to treat small soft tissue sarcomas.
Stereotactic radiotherapy treatment is given using a specially
adapted radiotherapy machine, sometimes called CyberKnife,
which delivers beams of radiotherapy from many different
angles. The beams overlap at the tumour, giving a very high
dose to it, but only a very small dose to surrounding tissues.
CyberKnife is only available in a small number of hospitals in
the UK. Your specialist can give you more information if this is
an option for you.
53
Understanding soft tissue sarcomas
Brachytherapy
Sometimes, radiotherapy is given internally by putting
radioactive material (the source) close to the tumour or the
area where the tumour was before surgery. This is called
brachytherapy. It’s not commonly used in the treatment of
soft tissue sarcomas.
Your specialist can discuss brachytherapy and its side effects
with you if they think it will be helpful.
Side effects of radiotherapy
You may develop the side effects listed on the following
pages over the course of your treatment. These side effects
will usually gradually disappear over a few weeks or months
after treatment is finished. Your doctor, nurse or radiographer
will discuss this with you so that you know what to expect.
Let them know about any side effects that you have during
or after treatment, as there are often things that can be done
to help.
Hair loss
Hair will only fall out in the area being treated by radiotherapy,
so the treatment for soft tissue sarcomas will not make the hair
on your head fall out. The hair that is lost may grow back after
the treatment has ended, but it is often lost permanently.
Skin care
Some people develop a skin reaction similar to sunburn.
Pale skin may become red and sore or itchy, and darker
skin may develop a blue or black tinge. Your radiographers
will give you advice on how to look after your skin.
54
Radiotherapy
Tiredness (fatigue)
This is a common side effect and may continue for months
after treatment finishes. During treatment, you’ll need to rest
more than usual, especially if you have to travel a long way
for treatment each day. But it’s good to do gentle exercise,
such as walking, when you feel able. Once your treatment is
over, gradually increase your activity and try to balance rest
periods with some physical activity. This will help you build up
your energy levels.
Feeling sick
Some people find that their treatment makes them feel sick
(nausea), and sometimes they may actually be sick (vomit).
This is most common when the treatment area is near the
stomach or bowel. If nausea and vomiting occur, they can
usually be effectively treated with anti-sickness drugs
(anti-emetics), which your doctor can prescribe. If you
don’t feel like eating, you can replace meals with nutritious,
high-calorie drinks, which are available from most chemists
and can be prescribed by your GP.
We can send you further information about each of
these side effects. Any side effects you have should
disappear gradually once your course of treatment
is over, but it’s important to let your doctor know if
they continue.
Radiotherapy doesn’t make you radioactive and
it’s perfectly safe for you to be with other people,
including children, throughout your treatment.
55
Understanding soft tissue sarcomas
Long-term side effects
After radiotherapy, some people may develop swelling known
as lymphoedema. This happens because the lymph nodes and
vessels can become damaged by the radiotherapy. Lymph fluid,
which circulates around the lymphatic system (see page 7),
is unable to pass along the vessels and builds up, causing
swelling. If lymphoedema develops, it can’t be cured but it
can often be treated and managed.
To help prevent lymphoedema, you should try to avoid getting
any infection or inflammation in the area that has been treated
with radiotherapy. You should try to avoid cuts or grazes in the
area and use a moisturiser if your skin gets dry.
Radiotherapy to a joint, such as the knee or elbow, may cause it
to become stiff. It’s important to keep the joint mobile by using
it and doing regular exercise to help prevent stiffness.
A small number of people will develop a second cancer
because of the radiotherapy treatment they’ve had. However,
the chance of a second cancer developing is so small that the
risks of having radiotherapy are far outweighed by the benefits.
To reduce the risk, radiotherapy is very carefully planned and
improvements in the way radiotherapy is given mean that the
risk of developing a second cancer is very small.
You can talk to your cancer specialist if you’re concerned about
your risk of developing a second cancer.
Our booklet Understanding radiotherapy gives detailed
information about this treatment and its side effects.
56
Chemotherapy
Chemotherapy
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to
destroy cancer cells. Chemotherapy drugs work by disrupting
the growth of cancer cells.
Chemotherapy may sometimes be given in the following ways:
•• Before surgery to shrink the tumour, making it easier
to remove. This is known as neo-adjuvant treatment.
•• After surgery to reduce the risk of the cancer coming back.
This is called adjuvant treatment.
•• To help control symptoms and improve a person’s quality
of life if the cancer can’t be removed or has spread to other
parts of the body. This is called palliative treatment.
In some people, the chemotherapy will achieve this.
Unfortunately, for other people, the chemotherapy will not
shrink the cancer and they will have the side effects of the
treatment with little benefit.
Chemotherapy is sometimes combined with radiotherapy
(see previous section).
Certain types of soft tissue sarcoma are always treated with
chemotherapy. For other types it is very rarely used. Your doctor
can tell you whether chemotherapy will be used to treat your
type of soft tissue sarcoma.
57
Chemotherapy
Giving chemotherapy
Chemotherapy drugs are usually given by injection into a vein
(intravenously) but can sometimes be given as tablets. The most
commonly used drugs for soft tissue sarcomas are:
•• doxorubicin
•• ifosfamide.
Other drugs which may be used include:
•• docetaxel
•• gemcitabine
•• paclitaxel
•• trabectedin
•• dacarbazine.
These drugs can be given on their own, or two or more may be
given together.
Chemotherapy is given as a session (or cycle) of treatment.
It may be given as an outpatient or you may need to be
admitted to hospital for a few days. Each treatment is followed
by a rest period of a few weeks to allow your body to recover
from any side effects. The number of cycles you have will
depend on the type of sarcoma you have and how well it’s
responding to the drugs.
Our booklet Understanding chemotherapy discusses this
treatment and its side effects in more detail. We also
have cancer information sheets about individual drugs
and their particular side effects, which we can send you.
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Understanding soft tissue sarcomas
Side effects of chemotherapy
Each person’s reaction to chemotherapy is different.
Some people have very few side effects while others may
experience more. The side effects described here won’t
affect everyone who is having this treatment.
We have outlined the most common side effects but haven’t
included those that are rare and unlikely to affect you. If you
do notice any effects that aren’t listed here, discuss them with
your doctor, chemotherapy nurse or pharmacist.
Risk of infection
Chemotherapy can reduce the number of white blood cells,
which help fight infection. White blood cells are produced
by the bone marrow. If your number of white blood cells is
low, you will be more prone to infections. A low white blood
cell count is called neutropenia. This begins a few days after
treatment. The number of white blood cells will then increase
steadily and usually return to normal before your next cycle
of chemotherapy is due.
Contact your doctor or the hospital straight away if:
•• your temperature goes above 38°C (100.4°F)
•• you suddenly feel unwell, even with a normal temperature.
You will have a blood test before having more chemotherapy
to check the number of white blood cells. Occasionally, your
treatment may need to be delayed if the number of blood cells
(blood count) is still low.
60
Chemotherapy
Bruising and bleeding
Chemotherapy can reduce the production of platelets,
which help the blood to clot. Tell your doctor if you notice
any unexplained bruising or bleeding, such as nosebleeds,
bleeding gums, blood spots or rashes on the skin. You can
have a platelet transfusion if your platelet count is low.
Anaemia (low number of red blood cells)
Chemotherapy can reduce the number of red blood cells,
which carry oxygen around the body. A low red blood
cell count is called anaemia. This may make you feel tired
and breathless. Tell your doctor or nurse if you have these
symptoms. You may need to have a blood transfusion if the
number of red blood cells becomes too low.
Feeling sick (nausea) or being sick (vomiting)
Your doctor can prescribe very effective anti-sickness
(anti-emetic) drugs to prevent, or greatly reduce, nausea
or vomiting. If the sickness isn’t controlled or if it continues,
tell your doctor. They can prescribe other anti-sickness drugs
that may be more effective.
Some anti-sickness drugs can cause constipation. Let your
doctor or nurse know if this is a problem for you.
Sore mouth
Your mouth may become sore or dry, or you may notice
small ulcers during this treatment. Drinking plenty of fluids,
and cleaning your teeth regularly and gently with a soft
toothbrush, can help to reduce the risk of this happening.
Some people find sucking on ice soothing. Tell your nurse
or doctor if you have any of these problems, as they can
prescribe mouthwashes and medicine to prevent or clear
mouth infections.
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Understanding soft tissue sarcomas
Taste changes
Occasionally during treatment, you may get a strange, metallic
or bitter taste in your mouth. Some people find sucking on a
strongly flavoured sweet or mint helps to disguise this. You may
also notice that food tastes different, but your normal taste will
usually come back after treatment finishes.
Hair loss
Not all chemotherapy drugs cause hair loss. Hair may be lost
completely or may just thin. You can ask your doctor if the
drugs you are having are likely to cause hair loss.
It may be possible to reduce the amount of hair that you lose
by using scalp cooling. Cooling the scalp during chemotherapy
means that less chemotherapy reaches the hair follicles, and so
the hair is less likely to fall out. We can send you information
about scalp cooling.
If you do lose your hair, you may want to wear a wig.
You can ask your doctor or nurse to arrange for you to see a
wig specialist. You may prefer to wear a bandana, hat or scarf.
If your hair does fall out, it will almost always grow back over a
period of 3−6 months once the chemotherapy has finished.
Tiredness (fatigue)
Feeling tired is a common side effect of chemotherapy,
especially towards the end of treatment and for some weeks
after it’s over. It’s important to try to pace yourself and get as
much rest as you need. Try to balance this with taking some
gentle exercise, such as short walks, which will help. If tiredness
is making you feel sleepy, don’t drive or operate machinery.
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Chemotherapy
Side effects can be hard to deal with, but they usually
disappear gradually when your treatment finishes.
We can send you more detailed information about all
the side effects mentioned in this section. You can also
view this online at macmillan.org.uk/cancerinformation
Fertility
Your ability to become pregnant or father a child is likely to
be affected by having chemotherapy. It’s important to discuss
fertility with your doctor before starting treatment, as it may be
possible for men to store sperm and women to store eggs or
embryos for use in the future. We can send you more detailed
information about fertility and cancer treatment.
Some women may find that chemotherapy treatment causes an
early menopause, and they may have symptoms such as hot
flushes and sweats. In many cases, HRT (hormone replacement
therapy) can be given to replace the hormones that are no
longer being produced. Women with gynaecological sarcomas
may not be able to have HRT because the cancer may be
sensitive to hormones.
You may find it helpful to talk all this through with your doctor
or one of the support organisations listed on pages 91–97.
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Understanding soft tissue sarcomas
Contraception
It’s not advisable to become pregnant or father a child while
having chemotherapy as it may harm the developing baby.
It’s important to use effective contraception during your
treatment and for at least a few months afterwards.
You can discuss this with your doctor or nurse.
It’s not known whether chemotherapy drugs can be present
in semen or vaginal fluids. To protect your partner, it’s safest
to either avoid sex or use a barrier form of contraception for
about 48 hours after chemotherapy.
64
Targeted therapies
Targeted therapies
Targeted or biological therapies are treatments that work by
targeting specific proteins that are found either on the surface
of cells or within the cell itself. They include cancer growth
inhibitors and monoclonal antibodies.
Targeted therapies are often given as part of cancer research
trials and are sometimes used to treat soft tissue sarcomas.
Imatinib (Glivec®)
Imatinib is a type of treatment called a tyrosine kinase inhibitor
(TKI). It may be used to treat GISTs (see page 13), as the
effects of the drug are very specific to this type of sarcoma.
Imatinib works by blocking (inhibiting) signals within cancer
cells. Blocking the signals prevents a series of chemical
reactions that make the cells grow and divide. The chemical
it blocks is called tyrosine kinase.
Imatinib is taken once a day as tablets. It can cause side effects
including feeling sick (nausea), vomiting, diarrhoea, a skin rash
and puffiness, especially around the eyes. Some of the side
effects can be reduced with other medicines. Imatinib can
sometimes control GISTs for several years.
Sunitinib (Sutent®)
Sunitinib is another TKI. It may be used to treat GISTs that
don’t respond to imatinib, or if the side effects of imatinib
are too troublesome.
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Understanding soft tissue sarcomas
As well as blocking signals within the cancer cells and
preventing a series of chemical reactions that make the cell
grow and divide, it helps to prevent the tumour developing new
blood vessels. Without blood vessels, the tumour can’t get the
nutrients it needs to survive.
Possible side effects of sunitinib include tiredness, diarrhoea,
sore hands and feet, a sore mouth, taste changes and a loss
of appetite.
Pazopanib (Votrient®)
This is a newer TKI that is used to treat some types of advanced
soft tissue sarcoma. It is taken once a day as tablets.
Pazopanib has not been approved by the National Institute for
Health and Clinical Excellence (NICE) or the Scottish Medicines
Consortium (SMC) for use in the NHS. As a result, it may not
be widely available.
We can give you more information on what you can do
if a treatment isn’t available.
66
After treatment – follow-up
After treatment – follow-up
While most people recover well after surgery for soft
tissue sarcomas, and are able to move around quite soon,
some people take longer and need extra help. Before you
go home, the hospital staff will talk to you about your situation.
If you live alone or have stairs to climb, you may need some
help at home (see page 78).
If you have any worries about going home, make sure you
discuss them with the hospital staff in advance so that help
can be organised.
Children and teenagers often worry about falling behind
at school. Home tutoring can often be arranged with the local
education authority. The social worker on your ward will be
able to organise this for you.
After your treatment has finished, your doctor will want you to
have regular check-ups and x-rays, in particular chest x-rays.
These will often continue for several years. If you have any
problems or notice any new symptoms in between these times
(for example, a lump or swelling at the area of your operation),
let your doctor know as soon as possible.
The gaps between your appointments will get longer as time
goes on. This is because the risk of the cancer coming back
gets steadily lower over time.
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Understanding soft tissue sarcomas
Share your experience
When treatment finishes, many people find it helps to talk
about it and share their thoughts, feelings and advice with
other people.
This can be especially helpful for other people with a soft
tissue sarcoma who are perhaps about to start their treatment.
Just hearing about how you’ve coped, what side effects you
had and how you managed them is very helpful to someone
in a similar situation.
We can help you share your story. Call us on 0808 808
00 00 or visit our website macmillan.org.uk for more
information about becoming a Cancer Voice.
If the cancer comes back
Sometimes, the sarcoma may come back or spread to other
parts of the body including, most often, the lungs. If tests show
that you have a small amount of cancer in your lungs, it may
be possible for you to have an operation to remove the part of
the lung that is affected (see page 38).
If the cancer comes back elsewhere, your doctor will talk to you
about the different treatment options available to you. The most
common treatment for people in this situation is chemotherapy
(see pages 57–64).
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Understanding soft tissue sarcomas
Research – clinical trials
Cancer research trials are carried out to try to find new and
better treatments for cancer. Trials that are carried out on
patients are called clinical trials. These may be carried out to:
•• test new treatments, such as new chemotherapy drugs,
gene therapy or cancer vaccines
•• look at new combinations of existing treatments, or change
the way they are given, to make them more effective or to
reduce side effects
•• compare the effectiveness of drugs used to control symptoms
•• find out how cancer treatments work
•• see which treatments are the most cost-effective.
Trials are the only reliable way to find out if a different
operation, type of chemotherapy, radiotherapy or other
treatment is better than what is already available.
Taking part in a trial
You may be asked to take part in a treatment research trial.
There can be many benefits in doing this. Trials help to improve
knowledge about cancer and develop new treatments. You will
be carefully monitored during and after the study.
Usually, several hospitals around the country take part in these
trials. It’s important to bear in mind that some treatments that
look promising at first are often later found not to be as good
as existing treatments or to have side effects that outweigh
the benefits.
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Research – clinical trials
If you decide not to take part in a trial, your decision will be
respected and you don’t have to give a reason. However, it can
help to let the staff know your concerns so that they can give
you the best advice. There will be no change in the way that
you’re treated by the hospital staff, and you’ll be offered the
standard treatment for your situation.
Blood and tumour samples
Blood and bone marrow or tumour samples may be taken
to help make the right diagnosis. You may be asked for your
permission to use some of your samples for research into
cancer. If you are taking part in a trial you may also be asked
to give other samples which may be frozen and stored for
future use, when new research techniques become available.
These samples will have your name removed from them so you
can’t be identified.
The research may be carried out at the hospital where you
are treated, or at another one. This type of research takes a
long time, and results may not be available for many years.
The samples will, however, be used to increase knowledge
about the causes of cancer and its treatment. This research will
hopefully improve the outlook for future patients.
Current research
A number of research trials are looking at whether different
combinations of chemotherapy or targeted therapies can help
to control the growth of some soft tissue sarcomas that have
spread. Your cancer specialist will be able to tell you more
about these trials. Our website macmillan.org.uk also has
links to clinical trial databases.
71
Your feelings
Your feelings
Most people feel overwhelmed when they are told they have
cancer, and have many different emotions. These are part of
the process you may go through when dealing with your illness.
Partners, family members and friends often have similar feelings
and may also need support and guidance to help them cope.
Reactions differ from one person to another – there’s no
right or wrong way to feel. We describe some of the common
emotional effects of cancer here. However, reactions vary and
people have different emotions at different times.
Our booklet How are you feeling? discusses the
emotions you may have in more detail, and has
suggestions for coping with them.
Shock and disbelief
Disbelief is often the immediate reaction when cancer is
diagnosed. You may feel numb and unable to express any
emotion. You may also find that you can only take in a small
amount of information, and so you have to keep asking the
same questions again and again. This need for repetition is a
common reaction to shock. Some people find that their feelings
of disbelief make it difficult for them to talk about their illness
with family and friends. For others it may be the main topic of
conversation as it’s the main thing on their mind.
You may find our booklet Talking about your
cancer helpful.
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Understanding soft tissue sarcomas
Fear and uncertainty
Cancer is a frightening word surrounded by fears and myths.
One of the greatest fears people have is whether they will die.
Many cancers are curable if found at an early stage. When a
cancer is not curable, current treatments often mean that it can
be controlled for some time.
Many people are anxious about whether their treatment
will work and have any side effects. It’s best to discuss your
treatment and possible outcomes in detail with your doctor.
You may find that doctors can’t answer your questions fully,
or that their answers sound vague. It’s often impossible for
them to say for certain how effective treatment will be.
Doctors know roughly how many people may benefit from
a certain treatment, but they can’t predict the future for a
particular person. Many people find this uncertainty hard to
live with, but your fears may be worse than the reality. Finding
out about your illness can be reassuring. Discussing what you
have found out with your family and friends can also help.
You might find it helpful to talk to other people in your situation.
Call our cancer support specialists on 0808 808 00 00 to find
out if there’s a support group in your area. Or you can visit our
online community at macmillan.org.uk/community to chat
any time with people who know what you’re going through.
Some people find some form of spiritual support helpful at this
time, and you may like to talk to a spiritual or religious adviser.
74
Your feelings
Denial
Many people cope with their illness by not wanting to know
much or talk much about it. If that’s the way you feel, just let
your family and friends know that you’d prefer not to talk about
your illness, at least for the time being.
Sometimes, however, it’s the other way around. You may
find that your family and friends don’t want to talk about your
illness. They may appear to ignore the fact that you have
cancer, perhaps by playing down your worries and symptoms
or deliberately changing the subject. If this upsets or hurts you, try telling them. Perhaps start by reassuring them that you know
why they’re doing it, but that it will help you if you can talk to
them about your illness.
Anger
People often feel very angry about their illness. Anger can also
hide other feelings, such as fear or sadness. You may direct
your anger at the people closest to you, or at the doctors and
nurses caring for you. It’s understandable that you may be very
upset by many aspects of your illness, so you don’t need to feel
guilty about your angry thoughts or irritable moods.
Bear in mind that your family and friends may sometimes think
that your anger is directed at them, when it’s really directed at
your illness. It may help to tell them this, or perhaps show them
this section of the booklet.
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Understanding soft tissue sarcomas
Blame and guilt
Sometimes, people blame themselves or others for their illness,
trying to find reasons to explain why it has happened to them.
This may be because we often feel better if we know why
something has happened. In most cases, it’s impossible to
know exactly what has caused a person’s cancer. So there’s
no reason for you to feel that anyone is to blame.
Resentment
Understandably, you may feel resentful because you have
cancer while other people are well. These feelings may crop
up from time to time during the course of your illness and
treatment. It usually helps to discuss these feelings, rather than
keeping them to yourself.
Isolation and depression
There may be times when you want to be left alone to work
through your thoughts and emotions. This can be hard for
your family and friends, who want to share this difficult time
with you. It may help them cope if you reassure them that,
although you don’t feel like discussing your illness at the
moment, you’ll talk to them about it when you’re ready.
Sometimes, depression can stop you wanting to talk. If you
or your family think you may be depressed, discuss this with
your GP. They can refer you to a doctor or counsellor who
specialises in the emotional problems of people with cancer,
or prescribe an antidepressant drug for you.
We have a video at macmillan.org.uk/depression that
you may find helpful.
76
What you can do
What you can do
One of the hardest things to cope with can be the feeling that
the cancer and its treatment have taken over your life. This is
a common feeling, but there are lots of things you can do.
There may be days when you feel too tired to even think about
what could help. You’ll have good and bad days, but if you’re
overwhelmed by these feelings, let your doctor or nurse know.
It may be that you have depression, and this is treatable so they
should be able to help.
Finding ways to cope
You may find it helps to try to carry on with life as normally as
possible, by staying in contact with friends and keeping up your
usual activities. Or you may want to decide on new priorities
in your life. This could mean spending more time with family,
going on the holiday you’ve dreamed about or taking up a new
hobby. Just thinking about these things and making plans can
help you realise that you still have choices.
Some people want to improve their general health by eating
a more healthy diet, by getting fitter or by finding a relaxing
complementary therapy.
Understanding the cancer and its treatment helps many people
cope. It means they can discuss plans for treatment, tests and
check-ups with their doctors and nurses. Being involved in these
choices can help give you back control of your life.
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Understanding soft tissue sarcomas
Who can help?
Many people are available to help you and your family.
District nurses work closely with GPs and make regular visits
to patients and their families at home if needed.
The hospital social worker can give you information about
social services and benefits you may be able to claim, such as
meals on wheels, a home helper or hospital fares. The social
worker may also be able to arrange childcare for you during
and after treatment.
In many areas of the country, there are also specialist nurses
called palliative care nurses. They are experienced in
assessing and treating symptoms of advanced cancer.
Palliative care nurses are sometimes known as Macmillan
nurses. However, many Macmillan professionals are nurses
who have specialist knowledge in a particular type of cancer.
You may meet them when you’re at a clinic or in hospital.
Marie Curie nurses help care for people approaching the end
of their lives in their own homes. Your GP or hospital specialist
nurse can usually arrange a visit by a palliative care or Marie
Curie nurse.
There’s also specialist help available to help you cope with the
emotional impact of cancer and its treatment. You can ask your
hospital doctor or GP to refer you to a doctor or counsellor who
specialises in supporting people with cancer and their families.
Our cancer support specialists on 0808 808 00 00 can
tell you more about counselling and can let you know about
services in your area.
78
If you are a relative or friend
If you are a relative or friend
Some people find it hard to talk about cancer or share their
feelings. You might think it’s best to pretend everything is fine,
and carry on as normal. You might not want to worry the
person with cancer, or you might feel you’re letting them down
if you admit to being afraid. Unfortunately, denying strong
emotions can make it even harder to talk, and may lead to
the person with cancer feeling very isolated.
Partners, relatives and friends can help by listening carefully to
what the person with cancer wants to say. It may be best not
to rush into talking about the illness. Often it’s enough just to
listen and let the person with cancer talk when they are ready.
You may find some of the courses on our Learn Zone website
helpful. There are courses to help with listening and talking,
to help friends and family support their loved ones affected by
cancer. Visit macmillan.org.uk/learnzone to find out more.
Our booklet Lost for words – how to talk to someone
with cancer has more suggestions if you have a friend
or relative with cancer.
If you’re looking after a family member or friend with cancer,
you may find our booklet Hello, and how are you? helpful.
It’s based on carers’ experiences and has lots of practical tips
and information.
We have more information about supporting someone
with cancer at macmillan.org.uk/carers
79
Relationships
Relationships
The experience of cancer may have improved your relationships
with people close to you. The support of family and friends may
have helped you cope. But cancer is stressful, and this can have
an effect on relationships. Any problems usually improve over
time, especially if you can talk openly about them.
Your partner
Some couples become closer through a cancer experience.
However, cancer can put a lot of strain on a relationship.
Problems sometimes develop, even between couples who’ve
been together for a long time. If a relationship was already
difficult, the stress of a major illness may make things worse.
Even couples that are close may not always know how their
partner is feeling. Talking openly about your feelings and
listening to each other can help you understand each other.
Our booklets Cancer, you and your partner and
Sexuality and cancer have more information that
may help.
Family and friends
Your family and friends may not always understand if you aren’t
feeling positive about getting on with things, and may not know
how big an effect treatment is having on your life. Talking about
how you feel will help them give you the support you need.
Our booklet Talking about your cancer has more
useful tips.
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Understanding soft tissue sarcomas
Talking to children
Deciding what to tell your children or grandchildren about your
cancer is difficult. An open, honest approach is usually best.
Even very young children can sense when something is wrong,
and their fears can sometimes be worse than the reality.
How much you tell your children will depend on their age
and how mature they are. It may be best to start by giving
only small amounts of information and gradually tell them
more to build up a picture of your illness.
Teenagers
Teenagers can have an especially hard time. At a stage when
they want more freedom, they may be asked to take on new
responsibilities and they may feel over-burdened. It’s important
that they can go on with their normal lives as much as possible
and still get the support they need.
If they find it hard to talk to you, you could encourage them
to talk to someone close who can support and listen to them,
such as a grandparent, family friend, teacher or counsellor.
They may also find it useful to look at the website riprap.org.uk
which has been developed especially for teenagers who have a
parent with cancer.
Our booklet Talking to children when an adult has
cancer includes discussion about sensitive topics.
There’s also a video on our website that may help,
at macmillan.org.uk/talkingtochildren
82
Financial help and benefits
Financial help and benefits
If you’re struggling to cope with the financial effects of cancer,
help is available.
If you’re an employee and unable to work because of illness,
you may be able to get Statutory Sick Pay. This is paid by
your employer for up to 28 weeks of sickness, and if you qualify
for it, your employer can’t pay you less.
Before your Statutory Sick Pay ends, check whether you can
claim Employment and Support Allowance. This benefit
gives financial help to people who are unable to work due to
illness or disability.
Disability Living Allowance (DLA) is for people under 65
who have difficulty walking or looking after themselves
(or both). You need to have had these difficulties for at least
three months to qualify, and they should be expected to last for
the next six months. As part of the Welfare Reform Act 2012,
DLA will be replaced by a Personal Independence Payment
for people of working age from April 2013.
Attendance Allowance (AA) is for people aged 65 or over
who have difficulty looking after themselves. You may qualify if
you need help with personal care, such as getting out of bed,
having a bath or dressing yourself. You don’t need to have a
carer to be eligible, but you must have needed care for at least
six months.
If you’re terminally ill, you can apply for DLA or AA under the
‘special rules’. This means your claim will be dealt with quickly
and you’ll receive the benefit you applied for at the highest rate.
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Understanding soft tissue sarcomas
Help for carers
If you are a carer, you may be entitled to financial help as well.
This may include Carer’s Allowance, which is the main state
benefit for carers, and Carer’s Credit, which helps carers build
up qualifying years for State Pension.
The benefits system can be complicated, so it’s a good
idea to talk to an experienced benefits adviser. You can
speak to one by calling the Macmillan Support Line on
0808 808 00 00. We’ve just outlined some benefits here,
but there may be others available to you.
You can find out more about benefits from Citizens Advice,
or by calling the Benefit Enquiry Line on 0800 882 200
(or 0800 220 674 if you live in Northern Ireland). The website
gov.uk (nidirect.gov.uk if you live in Northern Ireland) also
has useful information.
See our booklet Help with the cost of cancer for more
detailed information. Our video at macmillan.org.uk/
gettingfinancialhelp may also be useful.
Insurance
After having cancer treatment, it can be more difficult to get
certain types of insurance, including life and travel insurance.
An independent financial adviser (IFA) can help you assess your
financial needs and find the best deal for you. You can find an
IFA through one of the organisations listed on pages 93–95.
Our leaflet Getting travel insurance may be helpful.
84
Work
Work
You may need to take time off work during your treatment
and for a while afterwards. It can be hard to judge the best
time to go back to work, and this will depend mainly on the
type of work you do and how much your income is affected.
It’s important to do what’s right for you.
Getting back into your normal routine can be very helpful,
and you may want to go back to work as soon as possible.
It can be helpful to talk to your employer about the situation –
it may be possible for you to work part-time or job share.
On the other hand, it can take a long time to recover fully from
cancer treatment, and it may be many months before you feel
ready to return to work. It’s important not to take on too much,
too soon. Your consultant, GP or specialist nurse can help you
decide when and if you should go back to work.
Employment rights
The Equality Act 2010 protects anyone who has, or has had,
cancer. Even if a person who had cancer in the past has been
successfully treated and is now cured, they are still covered by
the act. This means their employer must not discriminate against
them for any reason, including their past cancer. The Disability
Discrimination Act protects people in Northern Ireland.
Our booklets Work and cancer, Working while caring for
someone with cancer and Self-employment and cancer
have more information that may be helpful. There’s also
lots more information at macmillan.org.uk/work
85
How we can help you
How we can help you
Cancer is the toughest fight most of us will
ever face. But you don’t have to go through
it alone. The Macmillan team is with you
every step of the way.
Get in touch
Macmillan Support Line
Our free, confidential phone
Macmillan Cancer Support line is open Monday–Friday,
89 Albert Embankment,
9am–8pm. Our cancer
London SE1 7UQ
support specialists provide
Questions about cancer?
clinical, financial, emotional
Call free on 0808 808 00 00 and practical information and
(Mon–Fri, 9am–8pm)
support to anyone affected by
www.macmillan.org.uk
cancer. Call us on 0808 808
Hard of hearing?
00 00 or email us via our
Use textphone
website, macmillan.org.uk/
0808 808 0121 or Text Relay. talktous
Non-English speaker?
Interpreters are available.
Information centres
Our information and support
Clear, reliable information centres are based in hospitals,
about cancer
libraries and mobile centres,
and offer you the opportunity
We can help you by phone,
to speak with someone
email, via our website and
face-to-face. Find your nearest
publications or in person.
one at macmillan.org.uk/
And our information is free to informationcentres
everyone affected by cancer.
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Understanding soft tissue sarcomas
Publications
We provide expert, up-to-date
information about different
types of cancer, tests and
treatments, and information
about living with and after
cancer. We can send you free
information in a variety of
formats, including booklets,
leaflets, cancer information
sheets, and audiobooks. We
can also provide information
in Braille and large print.
Need out-of-hours support?
You can find a lot of
information on our website,
macmillan.org.uk
For medical attention out of
hours, please contact your GP
for their out-of-hours service.
Someone to talk to
When you or someone you
know has cancer, it can be
difficult to talk about how
you’re feeling. You can call
our cancer support specialists
You can find all of our
information, along with several to talk about how you feel and
what’s worrying you.
videos, online at macmillan.
org.uk/cancerinformation
We can also help you find
support in your local area,
Review our information
so you can speak face-to-face
Help us make our resources
even better for people affected with people who understand
what you’re going through.
by cancer. Being one of our
reviewers gives you the chance
to comment on a variety of
information including booklets,
cancer information sheets,
leaflets, videos, illustrations
and website text.
If you’d like to hear more
about becoming a reviewer,
email reviewing@
macmillan.org.uk
88
How we can help you
Professional help
Support for each other
Our Macmillan nurses, doctors
and other health and social
care professionals offer expert
treatment and care. They help
individuals and families deal
with cancer from diagnosis
onwards, until they no longer
need this help.
No one knows more about
the impact cancer has on
a person’s life than those
who have been affected by
it themselves. That’s why we
help to bring people with
cancer and carers together in
their communities and online.
You can ask your GP, hospital
consultant, district nurse
or hospital ward sister if
there are any Macmillan
professionals available
in your area, or call us.
Support groups
You can find out about support
groups in your area by calling
us or by visiting macmillan.
org.uk/selfhelpandsupport
Online community
You can also share your
experiences, ask questions,
get and give support to
others in our online
community at macmillan.
org.uk/community
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Understanding soft tissue sarcomas
Financial and
work-related support
Find out more about the
financial and work-related
support we can offer
Having cancer can bring extra at macmillan.org.uk/
costs such as hospital parking, financialsupport
travel fares and higher heating
Learning about cancer
bills. Some people may have
to stop working.
You may find it useful to learn
If you’ve been affected in this more about cancer and how
to manage the impact it can
way, we can help. Call the
have on your life.
Macmillan Support Line and
one of our cancer support
You can do this online on our
specialists will tell you about
the benefits and other financial Learn Zone – macmillan.org.
uk/learnzone – which offers
help you may be entitled to.
a variety of e-learning courses
and workshops. There’s
We can also give you
information about your rights also a section dedicated to
supporting people with cancer
at work as an employee, and
help you find further support. – ideal for people who want
to learn more about what
their relative or friend is
Macmillan Grants
going through.
Money worries are the last
thing you need when you have
cancer. A Macmillan Grant is
a one-off payment for people
with cancer, to cover a variety
of practical needs including
heating bills, extra clothing,
or a much needed break.
90
Other useful organisations
Other useful organisations
Sarcoma support
organisations
General cancer
support organisations
Rarer Cancers Foundation
Unit 7B, Evelyn Court,
Grinstead Road,
London SE8 5AD
Helpline 0800 334 5551
Email
patientsupport@
rarercancers.org.uk
www.rarercancers.org.uk
Offers information to
people with rare cancers.
Puts people in touch with
others affected by a similar
cancer for mutual support.
Cancer Black Care
79 Acton Lane,
London NW10 8UT
Tel 020 8961 4151
Email
[email protected]
www.cancerblackcare.
org.uk
Offers information and
support for people with cancer
from ethnic communities, their
friends, carers and families.
Cancer Focus
Northern Ireland
Sarcoma UK
40–44 Eglantine Avenue,
49–51 East Road,
Belfast BT9 6DX
London N1 6AH
Tel 0800 783 3339
Tel 020 7250 8271
(Mon–Fri, 9am–1pm)
Email [email protected]
Email
www.sarcoma.org.uk
[email protected]
Offers support and information www.cancerfocusni.org
to anyone affected by
Offers a variety of services
sarcoma. The website has
to people affected by cancer,
information about various
including a free helpline,
types of sarcoma, as well
counselling and links to
as links to support groups
local support groups.
and helpful videos.
91
Understanding soft tissue sarcomas
Cancer Support Scotland Shelley Court, Gartnavel
Complex, Glasgow G12 0YN
Tel 0141 211 0122
Email info@
cancersupportscotland.org
www.
cancersupportscotland.org
Offers information
and support to people
affected by cancer. Also
runs support groups, and
provides counselling and
complementary therapies.
Email enquiries@
maggiescentres.org
www.maggiescentres.org
Maggie’s Centres provide
information about cancer,
benefits advice, and emotional
or psychological support. Penny Brohn Cancer Care
Chapel Pill Lane,
Pill, Bristol BS20 0HH
Tel 0845 123 2310
(Mon–Fri, 9.30am–5pm)
Email
[email protected]
Irish Cancer Society
www.
43–45 Northumberland Road, pennybrohncancercare.org
Dublin 4, Ireland
Offers a combination of
Tel 1800 200 700
physical, emotional and
(Mon–Thur, 9am–7pm,
spiritual support, using
Fri, 9am–5pm)
complementary therapies
Email [email protected] and self-help techniques.
www.cancer.ie
Tenovus
Has a freephone cancer
Head Office,
helpline staffed by nurses
Gleider House, Ty Glas Road,
trained in cancer care.
Cardiff CF14 5BD
You can also chat to a nurse
Tel 0808 808 1010
online and use the site’s
www.tenovus.org.uk
message board.
Provides a range of services
Maggie’s Centres
to people with cancer
1st Floor, One Waterloo Street, and their families,
Glasgow G2 6AY
including counselling
Tel 0300 123 1801
and a freephone helpline.
92
Other useful organisations
Counselling and
emotional support
Financial or legal
advice and information
British Association
for Counselling and
Psychotherapy (BACP) BACP House,
15 St John’s Business Park,
Lutterworth LE17 4HB
Tel 01455 883 300
Email [email protected]
www.bacp.co.uk
Promotes awareness of
counselling and signposts
people to appropriate services.
You can search for a qualified
counsellor at itsgoodtotalk.
org.uk
Benefit Enquiry Line
(England, Wales, Scotland)
Warbreck House, Warbreck
Hill Road, Blackpool FY2 0YE
Tel 0800 882 200 (Mon–Fri, 8.30am–6.30pm)
Email [email protected]
www.gov.uk/
benefit-enquiry-line
Provides advice and
information for disabled
people and carers on the
range of benefits available.
UK Council for
Psychotherapy (UKCP)
2nd Floor, Edward House,
2 Wakley Street,
London EC1V 7LT
Tel 020 7014 9955
Email [email protected]
www.psychotherapy.org.uk
Holds the national register
of psychotherapists and
psychotherapeutic counsellors,
listing practitioners who
meet exacting standards
and training requirements. NI Direct
(Northern Ireland)
Tel 0800 220 674
www.nidirect.gov.uk/
money-tax-and-benefits
Citizens Advice
Provides advice on a
variety of issues including
financial, legal, housing and
employment issues.
Find details for your local
office in the phone book or on
one of the following websites:
93
Understanding soft tissue sarcomas
England and Wales
www.citizensadvice.
org.uk
Scotland
www.cas.org.uk
Money Advice Scotland
Tel 0141 572 0237
www.moneyadvice
scotland.org.uk
National Debtline
(England, Wales
Northern Ireland
and Scotland)
www.citizensadvice.co.uk Tricorn House, 51–53 Hagley
Road, Edgbaston,
You can also find advice
Birmingham B16 8TP
online in a range of languages Tel 0808 808 4000
at adviceguide.org.uk
(Mon–Fri, 9am–9pm,
Sat, 9.30am–1pm)
Directgov
www.nationaldebtline.co.uk
www.gov.uk
A national helpline for
Has comprehensive
people with debt problems.
information about
The service is free, confidential
social security benefits
and independent.
and public services.
Personal Finance Society –
The Money Advice Service
‘Find an Adviser’ service
Holborn Centre, 120 Holborn, 42–48 High Road, South
London EC1N 2TD
Woodford, London E18 2JP
Tel 0300 500 5000
Tel 020 8530 0852
Email enquiries@
www.findanadviser.org
moneyadviceservice.org.uk
Use the website to find
www.moneyadvice
qualified financial advisers
service.org.uk
in your area.
Runs a free financial
health check service and
gives advice about
all types of financial matters.
94
Other useful organisations
Unbiased.co.uk
1 Sekforde Street,
London EC1R 0BE Email
[email protected]
www.unbiased.co.uk
On the website you can
search for qualified advisers
who specialise in giving
financial advice, mortgage,
accounting or legal advice.
Equipment and advice
on living with a disability
British Red Cross
UK Office,
44 Moorfields,
London EC2Y 9AL
Tel 0844 871 11 11
Email
[email protected]
www.redcross.org.uk
Offers a range of health and
social care services such as
care in the home, a medical
equipment loan service and
a transport service. Disability Rights UK
12 City Forum,
250 City Road,
London EC1V 8AF
Tel 020 7250 3222
Email enquiries@
disabilityrightsuk.org
www.disabilityrightsuk.org
Provides information on
social security benefits
and disability rights.
Disabled Living
Foundation (DLF)
380–384 Harrow Road,
London W9 2HU
Tel 0845 130 9177
(Mon–Fri, 10am–4pm)
Email [email protected]
www.dlf.org.uk
Provides free, impartial
advice about all types of
disability equipment and
mobility products.
Limbless Association
Unit 16, Waterhouse Business
Centre, 2 Cromar Way,
Chelmsford CM1 2QE
Helpline 0800 644 0185
Email [email protected]
www.limbless-association.
org
Offers support and
information to people who are
about to have an amputation
or those who are already living
with limb-loss.
95
Understanding soft tissue sarcomas
Support for young people Support for older people
CLIC Sargent
Horatio House, 77–85 Fulham
Palace Road, London W6 8JA
Tel 0300 330 0803
www.clicsargent.org.uk
Provides clinical, practical,
financial and emotional
support to children
with cancer.
Age UK
Tavis House, 1–6 Tavistock
Square, London WC1H 9NA
Tel (England and Wales)
0800 169 6565
Tel (Scotland)
0845 125 9732
Tel (Northern Ireland)
0808 808 7575
(Daily, 8am–7pm)
Teenage Cancer Trust
www.ageuk.org.uk
3rd Floor, 93 Newman Street,
Provides information and
London W1T 3EZ
advice for older people across
Tel 020 7612 0370
the UK via the website and
www.
advice line. Also publishes
teenagecancertrust.org
impartial and informative fact
A charity devoted to improving sheets and advice guides.
the lives of teenagers and
Support for carers
young adults with cancer. Runs a support network for
Carers Trust (Princess Royal
young people with cancer,
Trust for Carers in Scotland)
their friends and families.
32–36 Loman Street,
London SE1 0EH
Tel 0844 800 4361
Email [email protected]
www.carers.org
www.youngcarers.net
Provides support, information,
advice and services
for everyone caring at home
for a family member or friend.
96
Further resources
You can find details for
UK offices on the website.
Carers UK
20 Great Dover Street,
London SE1 4LX
Tel (England, Scotland,
Wales) 0808 808 7777
Tel (Northern Ireland)
028 9043 9843
(Wed–Thurs, 10am–12pm
and 2–4pm)
Email
[email protected]
www.carersuk.org
Offers information and
support to carers across
the UK. Can put people in
contact with support groups
for carers in their area.
You can search for
more organisations
on our website
macmillan.org.uk,
or call our support line
on 0808 808 00 00.
Further
resources
Related Macmillan
information
•• A parent’s guide to
children’s cancer
•• Cancer treatment and fertility
– information for men
•• Cancer treatment and fertility
– information for women
•• Cancer, you and
your partner
•• Controlling cancer pain
•• Coping with body changes
•• Coping with fatigue
•• Coping with hair loss
•• Getting travel insurance
•• Hello, and how are you?
•• Help with the cost of cancer
•• How are you feeling?
•• Lost for words – how to talk
to someone with cancer
97
Understanding soft tissue sarcomas
•• Relationships, sex and
fertility for young people
affected by cancer
•• Sexuality and cancer
•• Talking about your cancer
•• Talking to children when
an adult has cancer
•• Understanding cancer
research trials (clinical trials)
•• Understanding
chemotherapy
•• Understanding primary
bone cancer
•• Understanding radiotherapy
•• Work and cancer
•• Working while caring for
someone with cancer
To order, visit be.macmillan.
org.uk or call 0808 808 00
00. All of our information
is also available online
at macmillan.org.uk/
cancerinformation
To order the cancer
information sheets
mentioned in this booklet,
call 0808 808 00 00.
98
Macmillan audiobooks
Our high-quality audiobooks,
based on our variety of
booklets, include information
about cancer types, different
treatments and about living
with cancer.
To order your free CD,
visit be.macmillan.org.uk
or call 0808 808 00 00.
Useful websites
A lot of information
about cancer is available
on the internet. Some
websites are excellent;
others have misleading
or out-of-date information.
The sites listed here are
considered by nurses
and doctors to contain
accurate information and
are regularly updated.
Macmillan Cancer Support
www.macmillan.org.uk
Find out more about living
with the practical, emotional
and financial effects of cancer.
Further resources
Our website contains
expert, accurate, up-to-date
information about cancer and
its treatments, including:
•• all the information from our
150+ booklets and 360+
cancer information sheets
•• videos featuring real-life
stories from people affected
by cancer and information
from medical professionals
www.cancer.gov (National
Cancer Institute – National
Institute of Health – USA)
Gives comprehensive
information on cancer
and treatments.
www.cancerhelp.org.uk
(Cancer Research UK)
Contains patient information
on all types of cancer and has
a clinical trials database.
www.healthtalkonline.org
www.youthhealthtalk.org
(site for young people)
Both websites contain
•• how to contact our cancer
information about some
support specialists, including cancers and have video and
an email form to send
audio clips of people talking
your questions
about their experiences of
cancer and its treatments.
•• local support groups
search, links to other
www.macmillan.org.
cancer organisations
uk/cancerinformation/
and a directory of
teensandyoungadults
information materials
(Macmillan Cancer Support)
•• a huge online community of Macmillan’s cancer
people affected by cancer
information site written
sharing their experiences,
specifically for young people.
advice and support.
•• how Macmillan can help,
the services we offer and
where to get support
99
Understanding soft tissue sarcomas
www.macmillan.org.uk/
cancervoices (Macmillan
Cancer Voices) A UK-wide
network that enables people
who have or have had cancer,
and those close to them such
as family and carers, to speak
out about their experiences.
www.nhs.uk (NHS Choices)
NHS Choices is the online
‘front door’ to the NHS. It is
the country’s biggest health
website and gives all the
information you need to make
decisions about your health.
www.nhs24.com
(NHS 24 in Scotland)
www.nhsdirect.wales.nhs.uk
(NHS Direct Wales)
www.n-i.nhs.uk
(Health and Social Care
in Northern Ireland)
The official gateway to health
and social care services in
Northern Ireland.
www.patient.co.uk
(Patient UK) Provides people
in the UK with good-quality
information about health and
www.nhsdirect.nhs.uk
disease. Includes evidence(NHS Direct Online) NHS
based information leaflets on
health information site for
a wide variety of medical and
England – covers all aspects of health topics. Also reviews
health, illness and treatments. and links to many health- and
illness-related websites.
www.riprap.org.uk (Riprap)
Developed especially for
teenagers who have a parent
with cancer.
100
Your notes and questions
Your notes and questions
You could use these pages to write down any questions you
want to ask your doctor or nurse, and then to write down the
answers you receive.
101
Understanding soft tissue sarcomas
TNM staging
Two of the most commonly used staging systems
are a numbered staging system (see pages
28 –29) and the TNM staging system. The TNM
system is more detailed and is described here.
T stands for Tumour
Doctors put a number next to the ‘T’ to describe the size and
spread of the cancer.
There are two T stages for soft tissue sarcomas and each is
divided into ‘a’ and ‘b’:
T1 – The tumour is 5cm or less in size.
T1a – The tumour is near the surface of
the body (superficial).
T1b – The tumour is deep in the body.
T2 – The tumour is more than 5cm in size.
T2a – The tumour is superficial.
T2b – The tumour is deep in the body.
102
TNM staging
N stands for Nodes
This describes whether there are any lymph nodes near the
tumour that have cancer in them. The ‘N’ may have an ‘X’
or a number written next to it, which gives extra information
about the nodes that were examined:
NX – The lymph nodes were not examined.
N0 – The lymph nodes were examined but no cancer was found.
N1 – There are cancer cells in at least one lymph node.
If cancer cells have spread to the lymph nodes, the nodes are
said to be positive. It’s uncommon for soft tissue sarcomas to
spread to the lymph nodes. Positive nodes (N1) are classified
as a stage 3 sarcoma (see number staging on page 29).
M stands for Metastasis
Metastasis means that the cancer has spread to other parts of
the body such as the liver or lungs.
The ‘M’ may have a number written next to it, which gives extra
information about whether the cancer has spread or not:
M0 – The cancer has not spread to other parts of the body.
M1 – The cancer has spread to another part of the body.
This is called secondary or metastatic cancer.
103
Understanding soft tissue sarcomas
The grade of the cancer (see page 27) is also included in
the TNM staging system for soft tissue sarcomas:
G1 – low-grade
G2 – moderate- or intermediate-grade
G3 – high-grade
GX – the grade of the cancer isn’t known.
Our cancer support specialists on 0808 808 00 00
can tell you more about TNM staging. It’s also
important to talk to your doctor for detailed
information about your situation.
104
Disclaimer, thanks and sources
Disclaimer
We make every effort to ensure that the information we provide is accurate and up
to date but it should not be relied upon as a substitute for specialist professional
advice tailored to your situation. So far as is permitted by law, Macmillan does
not accept liability in relation to the use of any information contained in this
publication, or third-party information or websites included or referred to in it.
Thanks
This booklet has been written, revised and edited by Macmillan Cancer Support’s
Cancer Information Development team. It has been approved by our medical
editor, Dr Terry Priestman, Consultant Clinical Oncologist.
With thanks to: Laura Allington, Superintendent Radiographer; Dr Charlotte
Benson, Consultant Medical Oncologist; Dr Gail Horan, Clinical Oncologist;
Dr Aisha Miah, Consultant Clinical Oncologist; Dr Beatrice Sneddon, Consultant
Clinical Oncologist; Dr Jeremy Whelan, Consultant Medical Oncologist;
Dr Michael Williams, Clinical Oncologist; and the people affected by cancer
who reviewed this edition.
Sources
We’ve listed a sample of the sources used in this publication below.
If you’d like further information about the sources we use, please contact
us at [email protected]
Improving Outcomes for People with Sarcoma. March 2006. National Institute for
Health and Clinical Excellence (NICE).
UpToDate. Clinical presentation, histopathology, diagnostic evaluation, and staging
of soft tissue sarcoma. www.uptodate.com (accessed April 2012).
UpToDate. Local treatment for primary soft tissue sarcoma of the extremities and
chest wall. www.uptodate.com (accessed April 2012).
Weiss and Goldlum. Enzinger and Weiss’s Soft Tissue Tumours. 5th edition. 2008.
Mosby Elesevier.
105
Understanding soft tissue sarcomas
DeVita, et al. Cancer: Principles & Practice of Oncology. 8th edition. 2009.
Lippincott Williams and Wilkins.
Edge SB, et al. AJCC Cancer Staging Manual. 7th edition. 2010. Springer.
Trabectedin for the treatment of advanced soft tissue sarcoma. February 2010.
National Institute for Health and Clinical Excellence (NICE).
Sunitinib for the Treatment of Gastrointestinal Stromal Tumours. September 2009.
National Institute for Health and Clinical Excellence (NICE).
Grimer R, et al. UK Guidelines for the Management of Soft Tissue Sarcomas.
January 2010. British Sarcoma Group (submitted to Sarcoma).
106
Can you do something to help?
We hope this booklet has been useful to you. It’s just one of our
many publications that are available free to anyone affected by
cancer. They’re produced by our cancer information specialists
who, along with our nurses, benefits advisers, campaigners and
volunteers, are part of the Macmillan team. When people are
facing the toughest fight of their lives, we’re there to support
them every step of the way.
We want to make sure no one has to go through cancer alone,
so we need more people to help us. When the time is right for you,
here are some ways in which you can become a part of our team.
5 ways
you can someone
hElP with
cAncer
Share your cancer experience
Support people living with cancer by telling your story,
online, in the media or face to face.
Campaign for change
We need your help to make sure everyone gets the right support.
Take an action, big or small, for better cancer care.
Help someone in your community
A lift to an appointment. Help with the shopping.
Or just a cup of tea and a chat. Could you lend a hand?
Raise money
Whatever you like doing you can raise money to help.
Take part in one of our events or create your own.
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Cancer is the toughest fight most of us
will ever face. If you or a loved one has
been diagnosed, you need a team of
people in your corner, supporting you
every step of the way. That’s who we are.
We are the nurses and therapists helping you through
treatment. The experts on the end of the phone.
The advisers telling you which benefits you’re entitled to.
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things. The campaigners improving cancer care.
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You don’t have to face cancer alone.
We can give you the strength to get through it.
We are Macmillan Cancer Support.
Questions about living with cancer?
Call free on 0808 808 00 00 (Mon–Fri, 9am–8pm)
Alternatively, visit macmillan.org.uk
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© Macmillan Cancer Support, January 2013. 8th edition.
MAC11654. Next planned review 2015.
Macmillan Cancer Support, registered charity in England and
Wales (261017), Scotland (SC039907) and the Isle of Man (604).
Printed using sustainable material. Please recycle.