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Malignant Pleural Mesothelioma
Version 1.2012
Also available at NCCN.com
NCCN Guidelines for Patients
®
Presented with support from the national law firm of Baron & Budd
About this booklet
Its purpose
Learning that you have cancer can be overwhelming. The goal of this booklet
is to help you get the best cancer treatment. It explains which cancer tests and
treatments are recommended by experts of malignant pleural mesothelioma.
Supported by the NCCN Foundation
The NCCN Foundation supports the mission of the National Comprehensive
Cancer Network® (NCCN®) to improve the care of patients with cancer. One of its
aims is to raise funds to create a library of booklets for patients. Learn more about
the NCCN Foundation at www.nccn.com/nccn-foundation.
The source of the information
NCCN is a not-for-profit alliance of 21 of the world’s leading cancer centers.
Experts from NCCN have written treatment guidelines for doctors who treat
mesothelioma. These treatment guidelines suggest what the best practice is
for cancer care. The information in this booklet is based on these guidelines.
For more information
This booklet focuses on the treatment of mesothelioma. More information on
NCCN, mesothelioma, and other cancers can be found on NCCN.com.
© 2012 National Comprehensive Cancer Network, Inc. All rights reserved. The NCCN Guidelines for Patients™ and illustrations herein may not be reproduced in any form for any
purpose without the express written permission of NCCN.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
2
Table of contents
4
5
8
10
28
Part 6 – Supportive care
Part 2 – Am I at risk?
31
Part 7 – Accepting a treatment plan
Part 3 – Testing for mesothelioma
35
Part 8 – Tools
How to use this booklet
Part 1 – About mesothelioma
Explains where malignant pleural
mesothelioma starts and how it spreads.
Describes what increases your chances
for malignant pleural mesothelioma.
Explains when you should be tested and
the tests used.
15
Part 4 – Treatment planning
18
Part 5 – Treating mesothelioma
44
Describes the tests used to stage
mesothelioma and plan treatment.
51
56
Presents the recommended treatment
for malignant pleural mesothelioma.
21
S
tage I – III epithelioid
or mixed mesothelioma
27
S
tage I – III sarcomatoid
or Stage IV mesothelioma
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
3
Presents the recommended treatment
for the physical and emotional challenges
of mesothelioma.
Offers information to help you get a good
treatment plan.
Presents short reviews of Parts 1 to 7 along
with helpful webpages and questions to ask
your doctor.
Part 9 – Dictionary
The definitions of medical and other terms
are listed.
Credits
Index
How to use this booklet
Who should read this booklet?
The recommendations in this booklet include what the
NCCN doctors feel is the most useful based on science
and their experience. However, these recommendations
may not be right for you. Your doctors may suggest
other tests or treatments based on your medical history
and other factors. This booklet does not replace the
knowledge and suggestions of your doctors.
Mesothelioma is a rare cancer of the mesothelium—a
layer of cells that makes fluid. Mesothelioma can start
in the lining of the abdomen (peritoneal mesothelioma),
heart (pericardial mesothlioma), testicles (testicular
mesothelioma), or lungs (pleural mesothelioma). This
booklet focuses on mesothelioma that starts in the lining
of the lungs since it is the most common type. This
booklet may be helpful for patients, caregivers, and other
family and friends dealing with this cancer. Reading this
booklet at home may help you absorb what your doctors
have said and prepare for treatment.
Help! I don’t know these words!
In this booklet, many medical words are included that
describe cancer, tests, and treatments. These are words
that you will likely hear your treatment team use in the
months and years ahead. Most of the information may
be new to you, and it may be a lot to learn. Don’t be
discouraged as you read. Keep reading and review the
information.
Does the whole booklet apply to me?
Parts 1 and 2 review some basics about mesothelioma
that may help you understand the cancer better. If you’re
unsure if you should be tested for mesothelioma, read
Part 3. The information in Parts 4 through 8 is for people
who have mesothelioma. This information covers many
situations. Thus, not everyone will get every test and
treatment listed. Your treatment team can point out
what applies to you and give you more information.
As you read through this booklet, you may find it
helpful to create a list of questions to ask your doctors.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Words that you may not know are defined in the
text or the sidebar. Words with sidebar definitions are
underlined when first used on a page. All definitions are
listed in the Dictionary in Part 9. Acronyms are also listed
in the text or the sidebar. Acronyms are words formed
from the first letters of other words. One example is
U.S. for United States.
4
about
mesothelioma
Part 1: About mesothelioma
The lungs are covered by a double layer of thin tissue
called the pleura. The pleura protects and cushions the
lungs. Its inner layer of tissue is called the visceral pleura.
This layer is directly attached to the lungs and extends
down into the spaces between the lobes of the lungs.
The outer layer of the pleura is called the parietal pleura.
It lines the chest wall and has contact with many body
parts in the chest. See Figure 1. The chest wall is
the layer of muscles and bones under the skin in the
chest area.
The pleura is made of mesothelium and connective
tissue. Mesothelium is a single sheet of cells that
makes lubricating fluid. Connective tissue supports the
mesothelium and supplies it with blood. Connective
tissue attaches the pleura to other body parts.
There is space between the visceral and parietal pleura
called the pleural cavity. It is filled with a small amount of
pleural fluid made by the mesothelium. Pleural fluid acts
as a lubricant. It helps the two pleura layers slide against
each other during breathing. Plural fluid also helps the
lungs glide against other organs, such as the heart.
The same type of tissue as the pleura is found in other
areas of the body. However, it is called other names.
The tissue lining around the heart is called the
pericardium. The tissue lining around the belly
area between the chest and pelvis (abdomen) is
called the peritoneum. This booklet is about cancer
of the pleural mesothelium.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Figure 1. Pleural mesothelium
Illustration Copyright © 2012 Nucleus Medical Media, All rights reserved.
www.nucleusinc.com
6
Part 1
Part 2
Part 1: About mesothelioma
Part 3
Malignant pleural mesothelioma is the medical name
for cancer that starts in the mesothelium of the lungs.
It is also simply called mesothelioma. It isn’t lung cancer.
Lung cancer begins inside the lungs.
Part 4
Cancer is a disease of cells. Abnormal changes
(mutations) in genes can turn normal cells into cancer
cells. Genes are instructions in cells for making new cells
and controlling how cells behave. Gene mutations cause
cancer cells to act differently than normal cells.
Unlike normal cells, cancer cells can spread and form
tumors in other parts of the body. Cancer that has spread
is called a metastasis. Cancer cells often spread through
lymph or blood. Lymph is a clear fluid that gives cells
water and food. It also has white blood cells that fight
germs. Lymph nodes are groups of disease-fighting
cells that filter lymph and remove germs. Lymph travels
throughout the body in vessels like blood does.
As shown in Figure 2, lymph vessels and nodes are
found everywhere in the body.
Part 8
Illustration Copyright © 2012 Nucleus Medical Media, All rights reserved.
www.nucleusinc.com
7
Part 9
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Figure 2. Lymphatic system
Part 7
Part 6
Part 5
Normal cells grow and then divide to form new cells as
the body needs them. When normal cells grow old or get
damaged, they die. Cancer cells don’t do this. Cancer
cells make new cells that aren’t needed and don’t die
quickly when old or damaged. Over time, the cancer
cells grow and divide enough to form a mass called the
primary tumor. If the primary tumor isn’t treated, it can
grow and invade nearby tissues and organs, such
as the lungs.
am I
at risk?
Asbestos is the only known cause of
mesothelioma. Asbestos can break into tiny pieces
that may be breathed in or swallowed. The asbestos
pieces can then get trapped in the lungs and remain
there for years. How asbestos gets into the pleura (the
tissue lining around the lungs) and causes normal cells
to become cancer cells isn’t fully known. It often takes
20 to 40 years after having had contact with asbestos
for mesothelioma to develop. There are no studies that
clearly show the length of time exposed to asbestos that
is needed to increase the risk for mesothelioma.
Some patients with mesothelioma have had no clear
exposure to asbestos. This suggests that there may be
other causes of the disease. Prior radiation therapy, such
as that given for Hodgkin’s lymphoma, may be a cause.
Part 7
Part 6
Asbestos is divided into two main groups: serpentine
and amphibole asbestos. Serpentine is made of long,
curly fibers, whereas amphibole is made of straight,
needle-like fibers. There are different types of asbestos
within each main group, but the most commonly used
include chrysotile, crocidolite, and amosite.
9
Part 9
Part 8
Of these three, chrysotile is the most widely used
to make products. Chrysotile is a type of serpentine
asbestos. Its long, curly fibers make it less likely to
break into pieces and cause mesothelioma. Crocidolite
and amosite are types of amphibole asbestos. They are
used less frequently because their straight, needle-like
fibers are more brittle and likely to break, or fragment.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Part 2
Part 1
A gene mutation is an abnormal change in the
instructions for making and controlling cells. Recent
studies show that having a mutation in the BAP1 gene
may further increase the risk for mesothelioma in people
who have been exposed to asbestos. This gene mutation
can be passed down from parents to children.
Part 3
However, because they fragment, they are more likely to
be breathed in or swallowed and cause mesothelioma.
Part 4
Asbestos is a group of minerals made of tiny fibers. It is
strong, flexible, and resistant to heat and fire. Because
of these traits, asbestos has been used in housing and
commercial products, such as roofing and brake pads.
Part 5
Part 2: Am I at risk?
testing for
mesothelioma
• Trouble breathing,
• Chest CT (computed tomography) with contrast,
• Chest pain,
• Thoracentesis,
• A mass under the skin in the chest,
• Pleural biopsy, and
• Unplanned weight loss,
• Possible SMRP (serum mesothelin-related peptide)
blood test
• Excessive sweating,
• Lasting cough, and
Chest CT
A CT scan is a type of imaging test. Imaging tests take
pictures of the insides of your body. A CT scan takes
many pictures of a body part from different angles
using x-rays.
• Fever
Pleural effusion and thickening are two common signs
of asbestos exposure. Pleural effusion is the buildup of
extra fluid in the pleural cavity (the space between the
two pleura layers). It is often the earliest sign of asbestosrelated disease. Pleural thickening is widespread scarring
of the pleural tissue. Pleural plaques are concentrated
areas of pleural thickening. Pleural plaques are the most
common sign of asbestos-related disease, but are not
believed to lead to cancer.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
A CT of the chest is recommended for mesothelioma.
Your doctors will be able to see if tumors have formed
and the size of the tumors. They can also see if the
cancer has spread beyond the pleura.
A CT scan is easy to undergo. Before the test, you may
be asked to stop eating or drinking for several hours.
11
Part 1
Part 2
Part 3
Part 4
Part 5
Recommended tests
Part 6
Symptoms of mesothelioma
Part 7
To confirm if you have mesothelioma you will need
to be tested. The recommended tests are listed
and described next. These tests are used to find
mesothelioma, see how far the primary tumor has
spread, and plan treatment.
Part 8
It is recommended that you get tested for
mesothelioma if you have had pleural effusion
more than once, have pleural thickening, or both.
Testing for mesothelioma is done when certain symptoms
that may be caused by the cancer appear. Symptoms of
mesothelioma may not show until decades after contact
with asbestos. Symptoms of mesothelioma overlap with
symptoms of other health conditions, so only your doctor
will be able to tell if your symptoms are due to the cancer.
Possible symptoms of mesothelioma include, but aren’t
limited to:
Part 9
Part 3: Testing for mesothelioma
Part 3: Testing for mesothelioma
You also should remove any metal objects that are on
your body. You will likely be asked to change into a
hospital gown to wear during the test.
pleural cavity. See Figure 4. For thoracentesis, your
skin will be cleaned and numbed with local anesthesia.
Next, a needle will be inserted between your ribs into
your chest to get the fluid. You may be given an x-ray
before the biopsy to guide the needle into your chest and
afterward to check if the biopsy went well. The fluid will
then be sent to a lab to be tested for cancer cells and
biomarkers of cancer, such as cytokeratin.
A contrast dye may be used to make the pictures clearer.
The dye will be injected into your vein. It may cause
you to feel flushed or get hives. Rarely, serious allergic
reactions occur. Tell your doctor if you have had bad
reactions before.
As shown in Figure 3, a CT machine is large and has
a tunnel in the middle. During the test, you will need
to lie on a table that moves slowly through the tunnel.
Pillows or straps may be used to keep you still during the
test. You will be alone, but a technician will operate the
machine in a nearby room. He or she will be able to see,
hear, and speak with you at all times.
As the machine takes pictures, you may hear buzzing,
clicking, or whirring sounds. Earplugs are sometimes
worn to block these sounds. A computer combines all
pictures into one detailed picture. A CT scan is done in
about 30 seconds, but the entire process is completed
in 30 minutes. You may not learn of the results for a
few days since a radiologist needs to see the pictures.
Thoracentesis
To confirm if there’s cancer, a sample of tissue or fluid
must be removed from your body. The removal of a
tissue or fluid sample is called a biopsy. Thoracentesis
is a type of biopsy that removes a fluid sample from the
Figure 3. CT scan
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Local anesthesia: A
controlled loss of feeling
in a small area of the body
from drugs
Pleura: The tissue lining
around the lungs
Pleural cavity: The space
between the two pleura
layers
Figure 4. Thoracentesis
Illustration Copyright © 2012 Nucleus
Medical Media, All rights reserved.
www.nucleusinc.com
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Radiologist: A doctor
who’s an expert in reading
imaging tests
Part 4
Part 3
Part 2
Part 1
Cytokeratin: Type of
protein found on cells that
line the inside and outside
surfaces of the body
Part 5
Biomarker: A substance
found in body fluid or
tissues that may be a sign
of cancer
Part 6
Allergic reaction:
Symptoms caused when
the body is trying to rid
itself of invaders
Part 7
A CT-guided core biopsy uses pictures from a CT scan to guide a needle into
your body. First, your skin will be cleaned and numbed with local anesthesia.
Next, the needle will be inserted through a small cut and guided into your chest
between your ribs. Then, the sample will be removed by the sharp end of the
needle and stored in a hollow space within the needle.
Definitions
Part 8
Pleural biopsy
A pleural biopsy removes one or more samples of the pleura. The samples
are then sent to a lab to be tested for cancer cells and biomarkers of cancer,
such as cytokeratin. There are a few ways that a pleural biopsy can be done.
Options are a CT-guided core biopsy, an Abrams needle biopsy, an open
biopsy, or a thoracoscopic biopsy. Thoracoscopic biopsy is the preferred
method.
Part 9
Part 3: Testing for mesothelioma
Part 3: Testing for mesothelioma
An Abrams needle biopsy is like a core biopsy. However,
it may or may not use CT. An Abrams needle limits the
amount of air that enters tissue during a pleural biopsy.
An open biopsy requires a large surgical cut into the chest.
General anesthesia, a controlled loss of wakefulness from
drugs, is used. This cut allows the surgeon to see into
your chest without any tools. Samples are collected with a
surgical knife. For this biopsy, you will have to stay in the
hospital overnight.
For a thoracoscopic biopsy, also called a VATS (videoassisted thoracoscopic surgery) biopsy, a small cut into
your chest is needed. General anesthesia is used. Next,
a thoracoscope is inserted through the cut. A thoracoscope
is a thin, tube-shaped instrument that has a light and camera
for viewing as well as a tool to remove a sample. This
surgery may cause some pain and swelling and will leave
a small scar.
SMRP blood test
Mesothelin-related peptides are made when protein in the
mesothelium breaks down. Mesothelium is a single layer
of cells that makes lubricating fluid. These breakdown
products are found in blood (serum). People with
mesothelioma often have high levels of SMRP. This test
requires a sample of blood to be drawn from a vein in
your arm. The sample is then sent to the lab to be tested.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
14
treatment
planning
Part 4: Treatment planning
Chest and abdominal CT
If you have not already had a chest CT scan, it should
be done along with a CT of your abdomen. Contrast
should be used if possible. Contrast is a dye put into
your body to make clearer pictures during imaging tests.
These scans can show your doctors where the tumor is,
how big it is, and if it has spread. See page 11 for more
information about CT scans.
Based on the tests in Part 3, your doctors will let you
know if you have mesothelioma. If you do, a team of
experts with experience treating mesothelioma should
plan your treatment. Your team may include a:
• Pulmonologist - expert in treating lung diseases,
• Diagnostic imaging specialist - expert in imaging tests,
• Radiation oncologist - expert in radiation treatment,
• Medical oncologist - expert in cancer drugs, and
PET/CT
Like CT, PET is an imaging test that takes pictures of
the inside of your body. However, instead of showing
the shape of an organ or tumor, it shows how your cells
are using a simple form of sugar. To create the pictures,
a radiotracer first needs to be put into your body. A
radiotracer is matter with energy that is put into your
body to make pictures clearer. For mesothelioma, the
radiotracer used is FDG (18F-fluorodeoxyglucose). FDG
is made of fluoride and glucose (sugar). Cancer cells use
more FDG than normal cells and thus show up as bright
spots on pictures. For this test, you must fast for 4 hours
or more. PET is often used with CT. FDG PET/CT is
helpful for finding mesothelioma that has spread to lymph
nodes or distant sites. This test should be done before
talc pleurodesis. See page 29 for more information about
talc pleurodesis.
• Surgeon - expert in operations to remove or repair
a part of the body.
Once mesothelioma is confirmed, your doctors will need
to know the stage of the cancer. The cancer stage is a
rating by your doctors, based on tests, of how far the
cancer has grown and spread. It is used to plan which
treatments are best for you. The recommended tests for
cancer staging are:
Recommended tests
• Chest and abdominal CT with contrast,
• PET/CT (positron emission tomography/
computed tomography),
• Mediastinoscopy or EBUS-FNA (endobronchial
ultrasound–guided fine-needle aspiration),
Mediastinoscopy
If your treatment may include surgery, a biopsy of the lymph
nodes in the mediastinum is often recommended. The
mediastinum is the area of the chest between the lungs.
• Possible chest MRI (magnetic resonance imaging),
and
• Possible VATS and/or laparoscopy
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Diaphragm: A sheet of
muscles below the ribs that
helps a person to breathe
VATS and laparoscopy
VATS is a type of biopsy, also called thoracoscopic biopsy. At least two small cuts are
made between your ribs into your chest. A thoracoscope to see inside your chest is
inserted through one cut. Surgical tools are inserted through the other cuts to remove
samples of the pleura, lymph nodes, or other tissue. Laparoscopy is a type of surgery
to view and take biopsy samples of organs and tissue in the abdomen (belly). A
laparoscope is inserted through a tiny cut in your abdomen. A laparoscope is a thin,
lighted tube with a lens for viewing and tools to remove samples of tissue. These
procedures are recommended if imaging tests suggest that there is mesothelioma in
the pleura (tissue lining the lungs) on both sides of your chest or in the peritoneum
(tissue lining the abdomen).
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Lymph nodes: Small
groups of disease-fighting
cells located throughout the
body
Thoracoscope: Thin tube
with a light, lens, and tools
to see and remove samples
of tissue from the chest
Ultrasound: A test that
uses sound waves to take
pictures of inside the body
Part 3
Part 2
Part 1
MRI
MRI is an imaging test that uses radio waves and powerful magnets. It shows soft
tissues better than a CT scan. An MRI scan of your chest can show if the cancer has
spread to your chest wall, spine, diaphragm, or blood vessels. The scan may cause
your body to feel a bit warm. Like a CT scan, a contrast dye may be used.
Chest wall: The layer of
muscles and bones under
the skin covering the chest
Part 4
Bronchoscope: Thin, long
tube fitted with tools that is
guided down the throat
Part 5
EBUS-FNA
This is another way to perform a biopsy of the mediastinal lymph nodes. However,
EBUS-FNA doesn’t require any surgical cuts. During an EBUS-FNA, a bronchoscope
attached to an ultrasound device is guided down your throat into your lungs. The
ultrasound is used to find the lymph nodes. Next, a very thin needle is passed through
the wall of a main airway of the lungs and into a lymph node to get a sample.
Part 6
Biopsy: Removal of small
amounts of tissue or fluid to
be tested for disease
Part 7
Definitions
Part 8
Samples of these lymph nodes can be obtained by mediastinoscopy. For this biopsy, a
mediastinoscope is inserted through a small cut in your neck right above your sternum.
A mediastinoscope is a thin, tube-shaped instrument that has a light and camera for
viewing as well as a tool to remove a sample. General anesthesia is used. General
anesthesia is a controlled loss of wakefulness from drugs. This surgery may cause
some pain and swelling and will leave a small scar.
Part 9
Part 4: Treatment planning
treating
mesothelioma
The tumor has grown into either the diaphragm or the lung.
Stage III
• The tumor has grown into 1) the first layer of the chest wall
or a confined area of the other layers, 2) the fat between the
lungs, or 3) the pericardium, and/or
• The cancer has spread to nearby lymph nodes or the nodes
at the base of the breast bone.
Stage IV
• The tumor has grown into 1) a wide area of the chest wall, 2)
the peritoneum, 3) an organ between the lungs, 4) the spine,
or 5) the pleura around the other lung,
• The cancer has spread to the lymph nodes near the other
lung or near the collarbone, and/or
• The cancer has spread to distant sites.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Pericardium: The tissue
lining around the heart
Peritoneum: The tissue
lining around the organs in
the belly area
Pleura: The tissue lining
around the lungs
Spine: Bones, muscles,
and other tissues along the
back from the base of the
skull to the tailbone
Part 3
Part 2
Part 1
Lymph nodes: Small
groups of special diseasefighting cells located
throughout the body
Part 4
Stage II
Part 5
The tumor hasn’t grown beyond the pleura where it started.
Diaphragm: A sheet of
muscles below the ribs that
helps a person to breathe
Part 6
Stage I
Chest wall: The layer of
muscles and bones under
the skin covering the chest
Part 7
Stages of mesothelioma
Definitions
Part 8
The recommended treatment for mesothelioma is based on two features of the cancer.
One of these features is the clinical stage. The clinical stage is a rating of the extent
of a tumor based on tests before treatment. The AJCC (American Joint Committee on
Cancer) staging system is most often used for mesothelioma. In this system, there are
four cancer stages based on the growth and spread of the cancer described below:
Part 9
Part 5: Treating mesothelioma
Part 5: Treating mesothelioma
The second feature used to recommend treatment is the type of mesothelioma cell.
A pathologist will classify the cell type based on the size, shape, and structure of
the cells. This is known as histologic subtyping. The three types of mesothelioma
cells are:
Mesothelioma cell types
Epithelioid
The cells are uniform and form complex, organized patterns.
Sarcomatoid
The cells are spindle-shaped and form disorganized patterns.
Biphasic
(mixed)
The cells are both epithelioid and sarcomatoid cells.
Stages I – III with either an epithelioid or mixed cell type are treated the same. The
recommended treatments for these mesotheliomas can be found starting on page 21.
Stage IV mesothelioma and any mesothelioma with a sarcomatoid cell type are
treated the same. See page 27 for treatment recommendations.
Definitions
Definitions:
Artery: A tube that carries
blood from the heart to
organs throughout the body
Observation: A period of
testing to watch for when
to start treatment based on
cancer growth
Oxygen: A gas in the air
that the body needs to live
Pathologist: A doctor
who’s an expert in testing
cells to find disease
Radiotracer: Matter with
energy that is put into the
body to make pictures
clearer
Side effect: An unplanned
physical or emotional
response to treatment
Surgery: An operation to
remove or repair a part of
the body
Tumor: An overgrowth
of cells
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
20
Cardiac stress tests
Cardiac stress tests assess how well your heart works
while exercising. Some heart problems are easier to find
when your heart is working hard. Before the test, several
small patches will be placed on your chest. The patches
are connected to an ECG (electrocardiography) machine
by long wires. The ECG machine measures the electrical
activity of your heart during the test. A blood pressure cuff
may be fitted on your arm too. Next, your heart activity
will be recorded while you rest then as you exercise. The
level of exercise will be increased until you are unable to
keep going. After the test, you will be watched until your
heart activity and blood pressure return to normal.
• Possible perfusion scanning, and
• Cardiac stress test
Pulmonary function tests
These are tests that show how well your lungs work. You
may be asked not to eat much, not to smoke, and not to
take some medications before testing. A common side
effect of these tests is shortness of breath.
Spirometry involves blowing into a tube to measure how
much air and how fast you breathe. A gas diffusion test
involves breathing in a harmless gas and measuring how
much you breathe out. It tells how much oxygen travels
from your lungs into your blood. A body plethysmograph
involves sitting in a small room and breathing into a tube.
This test measures how much air your lungs can hold
and how much air is in your lungs after you exhale.
Next steps:
If surgery is still an option, read the next page for
treatment recommendations. If surgery isn’t possible,
you have two options. One option is to start observation
if you have no symptoms of the cancer. The second
option is to start treatment without delay. See page 27
to read about treatment options other than surgery.
Perfusion scanning
Perfusion scanning shows the blood flow in and out
of your lungs. You may have this test if the pulmonary
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
21
Part 1
Part 2
Part 3
Part 4
• Pulmonary function tests,
Part 5
Recommended tests
Part 6
function tests suggest your lungs aren’t working well.
First, a radiotracer will be injected into your arm. Next,
a camera that detects the radiotracer will take pictures
of your chest. In normal lungs, the radiotracer will be
evenly spread throughout the lungs. Areas without the
radiotracer may be caused by a blocked artery, tumor,
or other health problems.
Part 7
Surgery may be a treatment option. Your doctors will
use the tests described in Parts 3 and 4 to assess if
the tumor can be surgically removed. Also, your doctors
must assess the health of your heart and lungs.
Part 8
Stage I – III epithelioid or mixed mesothelioma
Part 9
Part 5: Treating mesothelioma
Part 5: Treating mesothelioma
Stage I – III epithelioid or mixed mesothelioma
Treatment with surgery
Definitions:
Neoadjuvant treatment
Tests
Primary treatment
Adjuvant treatment
Pemetrexed + cisplatin
• Chest CT, and
• Possible other
imaging tests
P/D (pleurectomy/decortication)
None
EPP (extrapleural pneumonectomy)
Radiation therapy
P/D (pleurectomy/decortication)
Chemotherapy
EPP (extrapleural pneumonectomy)
Chemotherapy +
radiation therapy
None
Neoadjuvant treatment
You can receive chemotherapy either before or after
surgery. Chemotherapy given before surgery is called
neoadjuvant treatment. It is given to shrink the tumor
before it is removed from the body. Pemetrexed with
cisplatin is recommended for neoadjuvant treatment.
Chemotherapy is given in cycles of treatment days
followed by days of rest. Cycles give the body a chance
to recover before the next treatment. The length of 1 cycle
of pemetrexed and cisplatin is 21 days. Pemetrexed and
cisplatin are given on Day 1 of the cycle. Both drugs are
liquids that are slowly injected into your vein.
Chemotherapy is the use of drugs to treat cancer.
Chemotherapy stops the growth process of cells in an
active growth phase. It doesn’t work on cells in a resting
phase. Cancer cells grow fast, so chemotherapy works
well to stop new cancer cells from being made. However,
it also kills normal cells and can cause side effects. Side
effects are unplanned physical or emotional responses to
treatment. Ask your doctor for a list of side effects caused
by chemotherapy.
Tests
If you receive neoadjuvant treatment, a CT scan of your
chest is recommended to check treatment results. The
CT scan can show if the tumor
is smaller and where it
Acronyms
is still present. See page 11 for more information on CT
scans. Based on the chest CT, your doctors may want
you to have another imaging test to assess if there’s
cancer in the area between your lungs.
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There are two types of surgery for mesothelioma: P/D (pleurectomy/
decortication) or EPP (extrapleural pneumonectomy). P/D removes the tumor
and the pleura affected by the tumor. EPP removes both pleural layers, the
lung and diaphragm on the same side of the chest as the tumor, and often
the pericardium. During both surgeries, lymph node sampling should be done
to assess if the cancer has spread to the lymph nodes between your lungs.
Lymph node sampling only removes some of the nodes from a cluster.
Lymph nodes: Small
groups of disease-fighting
cells located throughout the
body
There is no well-designed research to show which surgery is better in which
circumstances. Your doctors will recommend a surgery based on many factors.
They will consider your overall health, ability to do activities, symptoms,
cancer stage, and the type of mesothelioma cell. They will also consider that
the dangers of surgery are more likely and more serious with EPP than P/D. Ask
your treatment team for a list of all the dangers of the surgery they recommend.
Radiation therapy: The
use of radiation to treat
cancer
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Diaphragm: A sheet of
muscles below the ribs that
helps a person to breathe
Pericardium: The tissue
lining around the heart
Acronyms
CT = Computed
tomography
Part 3
Part 2
Part 1
Cancer stage: A rating of
the growth and spread of
the cancer
Part 4
Before surgery, your surgeon will perform a surgical exploration. This is a minor
surgery to look inside your chest to see how much the tumor has grown and
spread. Use of a thoracoscope inserted through one surgical cut is the preferred
method for exploration. A thoracoscope is a thin, tube-shaped instrument with
a light, camera lens, and tools on the end for viewing and to remove tissue from
the chest. If the whole tumor can’t be removed, surgery isn’t recommended.
Part 5
Board-certified: A status
to identify doctors who are
trained in a specialized field
of medicine
Part 6
Definitions
Part 7
Primary treatment
Primary treatment is the main treatment used to rid your body of cancer.
Surgery is used as a primary treatment for some mesotheliomas. Surgery
should be performed by a board-certified thoracic surgeon. A thoracic surgeon
is a doctor who’s an expert in operating on organs inside the chest.
Part 8
Stage I – III epithelioid or mixed mesothelioma
Part 9
Part 5: Treating mesothelioma
Part 5: Treating mesothelioma
Stage I – III epithelioid or mixed mesothelioma
Adjuvant treatment
Adjuvant treatment is given after surgery to treat any remaining cancer cells.
Adjuvant treatment after P/D is chemotherapy. If you had chemotherapy
before P/D, no adjuvant treatment is recommended. See page 27 for a list of
chemotherapy drugs for mesothelioma.
After EPP, radiation therapy is recommended. Chemotherapy may also be given if
not given before surgery. Radiation therapy uses high-energy rays to treat cancer.
The rays damage a cell’s instructions for creating and controlling cells. This either
kills the cancer cells or stops new cancer cells from being made. Radiation can
also harm normal cells. Ask your treatment team for a list of all the side effects
of radiation.
Radiation will be given to the side of your chest where the tumor was and other
areas decided by your radiation oncologist. For mesothelioma, radiation is often
given using a machine outside the body. This method is called EBRT (external
beam radiation therapy).
Notes
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
Before radiation, images of the chest are taken with a CT scan using contrast.
This process is called simulation. Your radiation oncologist will use the images to
decide the radiation dose and to shape the radiation beams. Beams are shaped
with computer software and hardware added to the radiation machine.
_____________________
Your treatment team will decide the best time to have radiation therapy
after surgery. During treatment, you will lie on a table in the same position
as done during simulation. Devices may be used to keep you from moving
so that the radiation targets the tumor. Likewise, methods may be applied
to control breathing.
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
_____________________
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Side effect: An unplanned
physical or emotional
response to treatment
Acronyms
CT = Computed
tomography
EPP = Extrapleural
pneumonectomy
P/D = Pleurectomy/
decortication
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Part 4
Part 3
Part 2
Part 1
Radiation oncologist:
A doctor who’s an expert
in treating cancer with
radiation
Part 5
Contrast: A dye put into
your body to make clearer
pictures during imaging
tests
Part 6
One session can take between 30 to 60 minutes. You will likely have 5 sessions
a week. Radiation therapy lasts 4 to 5 weeks if no cancer cells were found in the
normal-looking tissue around the tumor removed during surgery. If cancer is found,
radiation therapy lasts about 5 to 6 weeks.
Chemotherapy: Drugs that
stop the growth process
of cells in an active growth
phase
Part 7
You will be alone while the technician operates the machine from a nearby room.
He or she will be able to see, hear, and speak with you at all times. As treatment
is given, you may hear noises.
Definitions
Definitions:
Part 8
Stage I – III epithelioid or mixed mesothelioma
Part 9
Part 5: Treating mesothelioma
Part 5: Treating mesothelioma
Stage I – III epithelioid or mixed mesothelioma
The main types of EBRT used for mesothelioma include:
• 3D-CRT (three-dimensional conformal radiation therapy) – Radiation
is given in small doses for a few weeks with x-ray beams that match
the shape of the tumor,
• IMRT (intensity-modulated radiation therapy) – Radiation is given in small
doses for a few weeks with x-ray beams of different strengths based on the
thickness of the tumor,
• Tomotherapy – A type of IMRT, a tomotherapy machine takes CT scans and
delivers radiation, and
Definitions
Conformal radiation
therapy: Radiation beams
are shaped to match the
shape of the cancer using
imaging scans
EBRT: Radiation therapy
received from a machine
outside the body
• Proton beam therapy – Radiation is given using proton beams, which deliver
more radiation at the end rather than throughout the beam.
First-line treatment: The
first set of treatments given
to treat a disease
Conformal radiation therapy is the preferred method by NCCN doctors. IMRT,
tomotherapy, and proton beam therapy are newer radiation therapy methods.
They should only be used in experienced cancer centers or according to a specific,
detailed treatment plan called a protocol.
Second-line treatment:
The next treatment(s) given
when first-line treatment
fails
Side effect: An unplanned
physical or emotional
response to treatment
Acronyms
CT = Computed
tomography
EBRT = External beam
radiation therapy
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Chemotherapy
First-line treatment
Second-line treatment
Pemetrexed + cisplatin
(preferred)
Pemetrexed
Pemetrexed + carboplatin
Vinorelbine
Gemcitabine + cisplatin
The length of 1 cycle of gemcitabine and cisplatin is
21 to 28 days. For each cycle, gemcitabine is given on
Days 1, 8, and 15, and cisplatin is given only on Day 1.
Vinorelbine is given 1 time every 7 days.
If the cancer grows or spreads after first-line treatment,
then you may receive second-line treatment. However,
there is little research to provide guidelines for secondline treatment. If given as first-line treatment, pemetrexed
should only be given as second-line treatment if a good,
lasting response was achieved with it the first time.
Gemcitabine
Pemetrexed
Vinorelbine
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Part 1
Part 2
Part 3
Part 4
Part 5
Chemotherapy is given in cycles of treatment days
followed by days of rest. Cycles give the body a chance
to recover before the next treatment. The length of 1 cycle
of pemetrexed and cisplatin is 21 days. Pemetrexed
and cisplatin are given on Day 1 of the cycle. Other
treatments that also follow this cycle include pemetrexed
with carboplatin and pemetrexed alone.
Part 6
Cisplatin with pemetrexed is preferred for first-line
treatment. But, gemcitabine with cisplatin is a good
option if you cannot take pemetrexed. And pemetrexed
with carboplatin is a good option if you have poor overall
health, difficulty doing daily activities, or other serious
health conditions.
Part 7
Chemotherapy drugs stop the growth process of cells
in an active growth phase. Cancer cells grow fast, so
chemotherapy works well to stop new cancer cells from
being made. However, it also kills normal cells and can
cause side effects. Ask your doctor for a list of all the side
effects caused by the chemotherapy drugs you will have.
All of the chemotherapy drugs listed in the table below
are liquids that are slowly injected into your vein.
Part 8
Stage I – III sarcomatoid or Stage IV mesothelioma
Part 9
Part 5: Treating mesothelioma
supportive
care
• Distress
Pleural effusion
Pleural effusion is the buildup of extra fluid in the pleural cavity. This can result
in shortness of breath and make it hard to breathe. A pleural catheter can
be used to drain the fluid buildup. Another treatment is talc pleurodesis. Talc
pleurodesis involves putting talc powder into the pleural cavity. The powder
irritates the tissue and causes the pleura layers to grow together while healing.
This seals the pleural cavity and stops fluid buildup. It is important that a
PET/CT scan is done before talc pleurodesis because talc causes pleural
inflammation, which can look like cancer on the scan.
Chest pain
Chest pain may be caused by cancer growing into the chest wall. Radiation
therapy may be used to ease chest pain. However, there are other ways to
manage chest pain such as with pain medications or chemotherapy.
For more information about chest pain and management, talk to your
treatment team.
29
Pleura: The tissue lining
around the lungs
Pleural cavity: The space
between the two pleura
layers
Acronyms
PET/CT = Positron
emission tomography/
computed tomography
Part 4
Part 3
Part 2
Part 1
Inflammation: Redness,
heat, pain, and swelling
from injury or infection
• Smoking cessation, and
Part 5
• Chest pain,
Part 6
Chest wall: The layer of
muscles and bones under
the skin that covers the
chest area
• Pleural effusion,
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Catheter: A thin, flexible
tube used to give or drain
fluids from the body
Part 7
Conditions for supportive care
Definitions
Definitions:
Part 8
For most patients with cancer, their main concern is that their treatment works.
However, having cancer is complex and brings many physical and emotional
challenges. It is important to know about these challenges and get the support
you need. Supportive care is the treatment of symptoms of a disease. Part 6
lists some of the challenges you may face as well as ways to manage them.
Part 9
Part 6: Supportive care
Part 6: Supportive care
Smoking cessation
Smoking can worsen your overall health and how well
your lungs work. If you are a smoker, it is important to
talk with your treatment team about ways to quit. It may
also be very helpful to join a support group.
depression during a hard part of treatment. Feeling
distressed may be a minor problem, or it may be more
serious. Serious or not, tell your treatment team so that
you can get help if needed. Help can include support
groups, “talk” therapy, or medication. At your cancer
center, cancer navigators, social workers, and other
experts can help. Some people also feel better by
exercising, talking with loved ones, or relaxing.
Distress
Many patients with cancer experience symptoms of
distress, such as anxiety and depression. You may
feel anxious during testing, or you may have a passing
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accepting a
treatment plan
Part 7: Accepting a treatment plan
Parts of a treatment plan
Having cancer is very stressful. While absorbing the fact
that you have cancer, you must learn about tests and
treatments. In addition, the time you have to accept a
treatment plan feels short. Parts 1 through 6 aimed to
teach you about pleural mesothelioma. Part 7 addresses
issues related to accepting a treatment plan.
A treatment plan addresses all cancer care needs while
respecting your beliefs, wishes, and values. It is likely
to change and expand as you go through treatment.
The plan will include the role of your doctors and how
you can help yourself. A treatment plan often has the
following parts:
Benefits of a treatment plan
A treatment plan is a written course of action through
treatment and beyond. It can help you, your loved ones,
and your treatment team. A treatment plan is useful for:
Cancer information
Cancer can greatly differ even when people have a tumor
in the same organ. Test results that describe the cancer
are reported in the treatment plan. Such test results
include the cancer site, mesothelioma cell type, and
cancer stage.
• Starting and guiding talks about treatment,
• Teaching what the treatment choices are,
• Informing everyone of the decisions made,
Your treatment team
Pleural mesothelioma should be treated by a
multidisciplinary team with experience in mesothelioma.
Your treatment team may include a radiation oncologist,
thoracic surgeon, medical oncologist, diagnostic imaging
specialist, and pulmonologist. Your primary care doctor
can also be part of your team. Besides doctors, you
may receive care from nurses, social workers, and other
health experts. Ask to have the names and contact
information of your health care providers included
in the treatment plan.
• Pinpointing who is in charge of each part of care,
• Controlling stress,
• Knowing what to expect,
• Changing from one doctor to another,
• Improving contact among your doctors, and
• Providing care for all issues.
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• Treatment side effects, • Costs of treatment, and
• What you want from treatment.
The cancer treatment that you agree to have should be reported in the treatment plan.
It is also important to note the goal of treatment and the chance of a good treatment
result. In addition, all known side effects should be listed and the time required to treat
them should be noted.
Your treatment plan may change because of new information. You may change your
mind about treatment. Tests may find new results. How well the treatment is working
may change. Any of these changes may require a new treatment plan.
Stress and symptom control
Cancer and its treatment can cause bothersome symptoms. You may also have
symptoms from the stress of having cancer. Such symptoms include pain, sleep
loss, and anxiety. There are ways to treat many symptoms, so tell your treatment
team about any symptoms you have so they can help.
Financial stress is common. You may be unemployed or miss work during
treatment. You may have too little or no health insurance. Discuss these issues
with your treatment team and they will include information in the treatment plan
to help you control your finances.
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Pulmonologist: A doctor
who’s an expert in treating
lung diseases
Radiation oncologist:
A doctor who’s an expert
in treating cancer with
radiation
Side effect: An unplanned
physical or emotional
response to treatment
Thoracic surgeon: A
doctor who’s an expert in
operating on organs inside
the chest
Part 3
Part 2
Part 1
Medical oncologist: A
doctor who’s an expert in
cancer drugs
Part 4
• Your general health,
Part 5
Diagnostic imaging
specialist: A person
trained to read pictures of
the body from imaging tests
Part 6
• Location of the cancer,
Cancer stage: Rating of
the growth and spread of
the cancer
Part 7
• The cancer stage and cell type,
Definitions
Definitions:
Part 8
Cancer treatment
There is no single treatment practice that is best for all patients. There is often
more than one treatment option. Treatment planning takes into account many
factors, such as:
Part 9
Part 7: Accepting a treatment plan
Part 7: Accepting a treatment plan
Advanced care
Talking with your doctor about your prognosis can help
with treatment planning. If the cancer can’t be cured,
a care plan for the end of life can be made. However,
such talks often happen too late or not at all. Your doctor
may delay these talks for fear that you may lose hope,
become depressed, or have a shorter survival. Studies
suggest that these fears are wrong. Instead, there are
many benefits to advanced care planning. It is useful for:
Hospice care allows you to have the best quality of life
possible. Care is given all day, every day of the week. You
can choose to have hospice care at home or at a hospice
center. One study found that patients and caregivers had
a better quality of life when hospice care was started early.
An advance directive describes the treatment you’d want
if you weren’t able to make your wishes known. It also
can name a person whom you’d want to make decisions
for you. It is a legal paper that your doctors have to
follow. It can reveal your wishes about life-sustaining
machines, such as feeding tubes. It can also include
your treatment wishes if your heart or lungs were to stop
working. If you already have an advance directive, it may
need to be updated to be legally valid.
• Knowing what to expect,
• Making the most of your time,
• Lowering the stress of caregivers,
• Having your wishes followed,
• Having a better quality of life, and
Getting a 2nd opinion
• Getting good care.
The time around a cancer diagnosis is very stressful.
People with cancer often want to get treated as soon as
possible. While cancer can’t be ignored, there is time
to think about and choose which treatment plan is best
for you. You may wish to have another doctor review
your test results and the treatment plan your doctor has
recommended. This is called getting a 2nd opinion.
Advanced care planning starts with an honest talk between
you and your doctors. You don’t have to know the exact
details of your prognosis. Just having a general idea will help
with planning. With this information, you can decide at what
point you’d want to stop cancer treatment, if at all. You can
also decide what treatments you’d want for symptom relief.
Copies of all test results need to be sent to the doctor
giving the 2nd opinion. Some people feel uneasy asking
for copies from their doctors. However, a 2nd opinion is
a normal part of cancer care. What’s more, some health
plans require a 2nd opinion. If your health plan doesn’t
cover the cost of a 2nd opinion, you have the choice of
paying for it yourself.
Another part of the planning involves hospice care.
Hospice care doesn’t include treatment to fight the cancer
but rather to reduce symptoms caused by cancer. Hospice
care may be started because you aren’t interested in more
cancer treatment, no other cancer treatment is available,
or because you may be too sick for cancer treatment.
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Part 1
Review of Part 1 – About mesothelioma
• The pleural mesothelium is part of the tissue lining around the lungs.
• Malignant pleural mesothelioma is cancer that starts in the pleural mesothelium.
Part 3
Part 2
Part 8: Tools
American Cancer Society
www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-malignant-mesothelioma
National Cancer Institute
www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient/page1#Keypoint1
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Part 9
Part 8
Part 7
Mesothelioma Applied Research Foundation
www.curemeso.org/site/c.kkLUJ7MPKtH/b.8050629/k.6568/What_is_Mesothelioma.htm
Part 5
Webpages
Part 6
• Mesothelioma cells can spread to other body parts through lymph or blood.
Part 4
• Mesothelioma cells form a tumor since they don’t die as they should.
Part 8: Tools
Review of Part 2 – Risk factors
• Asbestos is a group of minerals made of tiny fibers that are strong and flexible.
• Asbestos can break into tiny pieces that may be breathed in or swallowed.
• Asbestos is the only known cause of mesothelioma.
Webpages
American Cancer Society
www.cancer.org/Cancer/MalignantMesothelioma/OverviewGuide/malignant-mesothelioma-overview-risk-factors
National Cancer Institute
www.cancer.gov/cancertopics/factsheet/Risk/asbestos
Mesothelioma Applied Research Foundation
www.curemeso.org/site/c.kkLUJ7MPKtH/b.8050905/k.7461/What_Causes_Mesothelioma.htm
CURE magazine
http://media.curetoday.com/downloads/documents/pocketguide_mesothelioma.pdf
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Part 1
• Pleural effusion is the buildup of extra fluid between the two layers of tissue lining the lungs.
• Pleural thickening is widespread scarring of the tissue around the lungs.
Part 2
Review of Part 3 – Testing for mesothelioma
Part 3
Part 8: Tools
American Cancer Society
www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-diagnosed
National Cancer Institute
www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient#Keypoint3
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Part 9
Part 8
Part 7
Mesothelioma Applied Research Foundation
www.curemeso.org/site/c.kkLUJ7MPKtH/b.8055879/k.705B/Mesothelioma_Diagnosis.htm
Part 5
Webpages
Part 6
• Imaging scans and tests of pleural fluid and tissue are needed to confirm if you
have mesothelioma.
Part 4
• Get tested for mesothelioma if you have had pleural effusion, pleural thickening, or both.
Part 8: Tools
Questions about testing to ask your doctor
• What tests will I have?
• Should I bring a list of my medications?
• Where will the tests take place?
Will I have to go to the hospital?
• Should I bring someone with me?
• How long will it take for me to recover?
Will I be given an antibiotic or other drug afterward?
• How long will it take? Will I be awake?
• Will it hurt? Will I need anesthesia?
• How soon will I know the results and who will
explain them to me? If a biopsy is done, will I
get a copy of the results?
• What are the risks? What are the chances of infection
or bleeding afterward?
• Who will talk with me about the next steps? When?
• How do I prepare for testing? Should I not take aspirin?
Should I not eat beforehand?
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Part 1
Review of Part 4 – Treatment planning
• Cancer staging is a rating of how far the cancer has grown and spread.
• The cancer stage is used to plan treatment.
Part 3
Part 2
Part 8: Tools
Part 4
• Cancer tests are used to determine the cancer stage.
National Cancer Institute
www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient/page2#Keypoint6
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Part 9
Part 8
Part 7
Mesothelioma Applied Research Foundation
www.curemeso.org/site/c.kkLUJ7MPKtH/b.8055915/k.3AAE/Mesothelioma_Staging_and_Outcomes.htm
Part 6
American Cancer Society
www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-staging
Part 5
Webpages
Part 8: Tools
Review of Part 5 – Treating mesothelioma
• Cancer treatment is based on the cancer stage and cell type.
• Chemotherapy is the use of drugs to treat cancer.
• Radiation therapy uses high-energy rays to treat cancer.
• There are 2 types of surgery for mesothelioma: P/D and EPP.
• Primary treatment is the main treatment used to rid your body of cancer.
• Neoadjuvant treatment is given before surgery; adjuvant treatment is given after surgery.
Webpages
American Cancer Society
www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-treating-by-extent
www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-treating-radiation
www.cancer.org/Cancer/MalignantMesothelioma/DetailedGuide/malignant-mesothelioma-treating-chemotherapy
National Cancer Institute
www.cancer.gov/cancertopics/pdq/treatment/malignantmesothelioma/patient/page4
Mesothelioma Applied Research Foundation
www.curemeso.org/site/c.kkLUJ7MPKtH/b.3081295/k.3C34/Pleural_Mesothelioma_Treatment.htm
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Part 1
• How much will the treatment cost? How can I find out
how much my insurance company will cover?
• How likely is it that I’ll be cancer-free after treatment?
• Where will I be treated? Will I have to stay in the
hospital or can I go home after each treatment?
• What symptoms should I look out for while being treated
for mesothelioma?
• What can I do to prepare for treatment?
• When will I be able to return to my normal activities?
• How many mesothelioma surgeries have you done?
How many of your patients have had complications?
• What is the chance that my cancer will come back and/
or spread?
• Is mesothelioma surgery a major part of your practice?
• What should I do after I finish treatment?
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Part 9
Part 8
• Will my age, general health, cancer stage, or other
medical conditions limit my treatment choices?
Part 4
• What are the risks and benefits of each treatment?
Part 5
• How soon should I start treatment? How long does
treatment take?
Part 6
• What are the available treatments for mesothelioma?
Do I have to get treated?
Part 7
Questions about treatment to ask your doctor
Part 3
Part 2
Part 8: Tools
Part 8: Tools
Review of Part 6 – Supportive care
• Supportive care is the treatment of symptoms of a disease.
• Talc pleurodesis or a pleural catheter can treat pleural effusion.
• Radiation therapy, chemotherapy, or pain drugs are used to treat chest pain.
• Talk to your doctor about ways to quit smoking and treatment for distress.
Webpages
American Cancer Society
www.cancer.org/Treatment/index
National Cancer Institute
www.cancer.gov/cancertopics/coping
NCCN
www.nccn.com/living-with-cancer.html
CURE magazine
http://media.curetoday.com/downloads/documents/pocketguide_mesothelioma.pdf
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Part 1
• A treatment plan can help you through cancer treatment and beyond.
• It covers many issues—test results, treatments, and supportive programs.
Part 2
Review of Part 7 – Accepting a treatment plan
Part 3
Part 8: Tools
Part 4
• You may wish to get a 2nd opinion on your treatment plan.
American Cancer Society
www.cancer.org/Treatment/FindingandPayingforTreatment/index
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
43
Part 9
Part 8
Part 7
Part 6
National Cancer Institute
www.cancer.gov/cancertopics/factsheet/Therapy/doctor-facility
Part 5
Webpages
Part 9: Dictionary
Abdomen
The belly area between the chest and
pelvis.
Asbestos
A group of minerals made of tiny
fibers that are strong, flexible, and
resistant to heat.
Body plethysmograph
A test that measures how much air
your lungs can hold and how much air
is in your lungs after you exhale.
Biomarker
A substance found in body fluid or
tissues that may be a sign of cancer.
Bronchoscope
A thin, long tube fitted with tools that
is guided down the throat.
Adjuvant treatment
A treatment given after the main
treatment used to cure disease.
Biopsy
Removal of small amounts of tissue or
fluid to be tested for disease.
Cancer staging
Ratings of the growth and spread of
tumors.
Allergic reaction
Symptoms caused when the body is
trying to rid itself of invaders.
Biphasic (mixed) subtype
A subtype of pleural mesothelioma
that has organized, structured cells
(epithelioid) and long, spindle-shaped,
disorganized cells (sarcomatoid).
Cardiac stress test
A test of how well your heart works
during exercise.
Abrams needle biopsy
A biopsy with a type of needle
designed to limit the amount of air that
enters tissue.
Amosite asbestos
Straight, brittle, needle-like asbestos
fibers.
Amphibole asbestos
A group of asbestos fibers that are
straight, needle-like, and brittle.
Anesthesia
Loss of feeling with or without loss of
wakefulness.
Blood vessel
A tube that circulates blood
throughout the body.
Board-certified
A status to identify doctors who
finished training in a specialized field
of medicine.
Artery
A tube that carries blood from the
heart to organs throughout the body.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Cells
The “building blocks” of tissues in
the body.
Chemotherapy
Drugs that stop the growth process of
cells in an active growth phase.
Chemotherapy cycle
Days of treatment followed by days
of rest.
Chest wall
The layer of muscles and bones under
the skin that covers the chest area.
44
Contrast
A dye put into your body to make
clearer pictures during imaging tests.
Core biopsy
Removal of a larger sample of tissue
using a wide needle to be tested for
disease.
Crocidolite asbestos
Straight, brittle, needle-like
asbestos fibers.
Diaphragm
A sheet of muscles below the ribs that
helps a person to breathe.
Distant metastasis
The spread of cancer cells from the
first tumor to a far site.
Electrocardiography (ECG)
machine
A machine that measures the
electrical activity of the heart during
exercise.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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External beam radiation therapy
(EBRT)
Radiation therapy received from a
machine outside the body.
Extrapleural pneumonectomy (EPP)
Surgery that removes the affected
lung and all its tissue lining, the sheet
of muscles below, and sometimes the
tissue lining around the heart.
First-line treatment
The first set of treatments given to
treat a disease.
Part 3
Part 2
Part 1
Diagnostic imaging specialist
A person trained to read pictures of
the body made by imaging tests.
Erionite
A mineral fiber similar to a type of
straight, brittle, needle-like asbestos
fiber.
Part 4
Diagnosis
To identify a disease.
Part 5
Conformal radiation therapy
Radiation beams are shaped to match
the shape of the cancer using imaging
scans.
Epithelioid subtype
The most common subtype of pleural
mesothelioma; epithelioid cells are
organized and structured.
Part 6
Computed tomography (CT) scan
A test that uses x-rays from many
angles to view a body part.
Cytokeratin
A type of protein found on cells that
line the inside and outside surfaces of
the body.
Endobronchial ultrasound–guided
fine-needle aspiration (EBUS-FNA)
Removal of tissue with a needle at the
end of a long, thin tube guided down
the main airway using imaging tests.
Part 7
Clinical stage
Rating of the extent of a tumor based
on tests before treatment.
CT-guided biopsy
Use of pictures from a CT scan to find
the right spot to remove a sample of
tissue or fluid to test for disease.
Part 8
Chrysotile asbestos
Long, curly asbestos fibers.
Part 9
Part 9: Dictionary
Part 9: Dictionary
18F-fluorodeoxyglucose (FDG)
A solution of fluoride and glucose
(sugar) put in your body to make
cancer show up better on certain
imaging tests.
Intensity-modulated radiation
therapy (IMRT)
Radiation therapy that uses small
beams of different strengths based on
the thickness of the tissue.
General anesthesia
A controlled loss of wakefulness
from drugs.
Lobe
A clearly seen division in an organ or
part of the body.
Histologic subtype
Grouping of cancer types based
on differences in the structure and
organization of cells.
Local anesthesia
A controlled loss of feeling in a small
area of the body from drugs.
Hives
Itchy, swollen, and red skin caused by
the body ridding itself of an invader.
Hospice care
Treatment only to reduce symptoms
caused by cancer without treating the
cancer itself.
Imaging tests
Tests that take pictures of the inside
of the body.
Inflammation
Redness, heat, pain, and swelling
from injury or infection.
Lung
An organ in the body made of airways
and air sacs.
Lymph vessels
Tube-shaped ducts that carry lymph
throughout the body.
Magnetic resonance imaging (MRI)
A test that uses radio waves and
powerful magnets to see the shape
and function of body parts.
Mediastinal lymph nodes
Groups of disease-fighting cells in the
middle of the chest.
Mediastinoscope
A thin, long tube fitted with tools to
work inside the chest.
Lymph
A clear fluid containing white blood
cells.
Mediastinoscopy
Use of a thin tool inserted above the
breastbone to do work in the middle of
the chest.
Lymph node sampling
Removal of one group of diseasefighting cells from a cluster.
Mediastinum
The area of the chest between the
lungs.
Lymph nodes
Small groups of special diseasefighting cells located throughout the
body.
Medical history
All health events and medications
taken to date.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Multidisciplinary team
A team that includes a number
of doctors and other health care
professionals who are experts in
different areas of cancer treatment.
Neoadjuvant treatment
The treatment given before the main
treatment used to cure disease.
Pathologist
A doctor who’s an expert in testing
cells to find disease.
Perfusion scanning
A test that assesses blood flow in and
out of the lungs.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Pleura
The tissue lining around the lungs.
Pleural biopsy
The removal of a sample of the
tissue lining around the lungs to
test for disease.
Pleural catheter
A tube that drains fluid from the chest.
Pleural cavity
The space between the two layers
of tissue lining around the lungs.
Pleural effusion
Excess fluid between the two layers
of tissue lining around the lungs.
Part 4
Part 3
Part 2
Part 1
Parietal pleura
The outer layer of the tissue lining
around the lungs.
Physical exam
A review of the body by a health
expert for signs of disease.
Part 5
Metastasis
The spread of cancer cells from the
first tumor to another body part.
Oxygen
A gas in the air that the body needs
to live.
Peritoneum
The tissue lining around the organs in
the belly area.
Part 6
Mesothelium
A single layer of cells that makes
lubricating fluid.
Open biopsy
A procedure that uses a surgical cut
to remove samples of tissue or fluid to
test for disease.
Pericardium
The tissue lining around the heart.
Part 7
Mesothelioma
A rare cancer that starts in the thin
layer of tissue that covers most
organs.
Observation
A period of testing right after treatment
to check that treatment worked; or
testing to monitor cancer to decide
when to start treatment.
Part 8
Medical oncologist
A doctor who’s an expert in
cancer drugs.
Part 9
Part 9: Dictionary
Part 9: Dictionary
Pleural fluid
The liquid in the space between
the two layers of the tissue lining
around the lungs.
Pleural mesothelioma
Cancer that starts in the tissue lining
around the lungs.
Pleural mesothelium
One layer of cells in the tissue
lining around the lungs that makes
lubricating fluid.
Pleural plaque
A concentrated area of scarring
in the tissue lining the lungs.
Pleural thickening
Widespread scarring in the tissue
lining the lungs.
Pleurectomy/decortication (P/D)
Surgery that removes the tumor and
affected part of the tissue lining the
lung.
Positron emission tomography
(PET)
A test that uses radioactive material
to see the function of body parts.
Primary treatment
The main treatment used to rid the
body of cancer.
Radiation oncologist
A doctor who’s an expert in treating
cancer with radiation.
Primary tumor
The first mass of cancer cells in
the body.
Radiation therapy
The use of radiation to treat cancer.
Prognosis
The course and outcome of a disease.
Radiologist
A doctor who’s an expert in reading
imaging tests.
Progression
When cancer grows or spreads in the
body.
Radiotracer
Matter with energy that is put into the
body to make pictures clearer.
Protocol
A detailed plan of a medical study,
treatment, or procedure.
Recurrence
The return of cancer after treatment.
Proton beam therapy
Radiation therapy that uses protons to
treat a disease.
Pulmonary function tests
A set of breathing tests to test the
strength of the lungs.
Pulmonologist
A doctor who’s an expert in treating
lung diseases.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Risk factor
Something that increases the chance
of getting a disease.
Sarcomatoid subtype
The type of pleural mesothelioma with
random and irregular cell shapes.
Second-line treatment
The next treatment(s) given when
first-line treatment fails.
Part 1
Surgery
An operation to remove or repair a
part of the body.
Simulation
The steps needed to prepare for
radiation therapy.
Surgical exploration
A procedure that looks inside your
body to assess the extent of the
cancer to see if surgery is possible.
Single agent
The use of one drug.
Spine
The bones, muscles, and other
tissues along the back from the base
of the skull to the tailbone.
Talc pleurodesis
A medical procedure that puts powder
in the area between the two layers
of tissue lining the lungs to seal the
layers together to stop fluid buildup.
Spirometry
A test that uses a tube to measure
how fast you breathe.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Thoracoscope
A thin, lighted tube with a lens and
tools to view and remove samples of
tissue from the chest.
Thoracoscopic biopsy
Use of thin tools inserted through a
small cut in the chest to remove tissue
to test for disease. Also called VATS
biopsy.
Three-dimensional conformal
radiation therapy (3D-CRT)
Radiation therapy that uses beams
that match the shape of the tumor.
Tomotherapy
A type of intensity-modulated radiation
therapy that takes images of the
tumor and delivers radiation.
Part 2
Side effect
An unplanned physical or emotional
response to treatment.
Surgeon
A doctor who’s an expert in operations
to remove or repair a part of the body.
Part 3
Thoracic surgeon
A doctor who’s an expert in operating
on organs inside the chest.
Part 4
Supportive care
Treatment for symptoms of a disease.
Part 5
Serum mesothelin-related peptide
(SMRP)
A protein in cells of the mesothelium
(the tissue lining of most organs in the
body) that can be measured in the
blood.
Part 6
Thoracentesis
Use of a needle inserted between the
ribs to remove fluid around the lungs.
Part 7
Sternum
A flat bone in the center of the chest;
also called the breastbone.
Part 8
Serpentine asbestos
A group of asbestos fibers that are
long, curly, and unlikely to break.
Part 9
Part 9: Dictionary
Part 9: Dictionary
Treatment plan
A written course of action through
cancer treatment and beyond.
Tumor
An overgrowth of cells.
Ultrasound
A test that uses sound waves to take
pictures of the inside of the body.
Video-assisted thoracoscopic
surgery (VATS)
Use of thin tools inserted between the
ribs to do work in the chest.
Visceral pleura
The inner layer of tissue lining around
the lungs.
White blood cell
A type of blood cell that fights
infection. NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Credits
NCCN aims to improve the care given to patients
with cancer. NCCN staff work with experts to create
helpful programs and resources for many stakeholders.
Stakeholders include health care providers, patients,
businesses, and others. One resource is the series of
booklets for patients called the NCCN Patient Guidelines.
Each booklet presents the standard of care for a type
of cancer.
NCCN Patient Guidelines
NCCN abbreviations and acronyms
Laura J. Hanisch, PsyD
Medical Writer/
Patient Information Specialist
The people involved in the making of the guidelines
for patients and doctors are listed next, starting with
NCCN staff:
Dorothy A. Shead, MS
Director
Patient and Clinical Information Operations
NCCN®
National Comprehensive Cancer Network®
Lacey Marlow
Associate Medical Writer
NCCN Patient Guidelines™
NCCN Guidelines for Patients™
NCCN Guidelines
NCCN Guidelines®
NCCN Clinical Practice Guidelines in Oncology®
Kristina M. Gregory, RN, MSN, OCN
Vice President/
Clinical Information Operations
The patient booklets are based on guidelines written
for doctors. These guidelines are called the NCCN
Guidelines. They give a step-by-step course of care that
many cancer doctors follow. Panels of experts create
the NCCN Guidelines. Most of the experts are from the
21 NCCN Member Institutions. Panelists may include
surgeons, radiation oncologists, medical oncologists,
and patient advocates. Recommendations in the NCCN
Guidelines are based on clinical trials and the experience
of the panelists.
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Miranda Hughes, PhD
Oncology Scientist/
Senior Medical Writer
NCCN Marketing
Rene Dubnanski
Graphic Design Specialist
51
NCCN Panel Members for Malignant Pleural Mesothelioma
David S. Ettinger, MD
The Sidney Kimmel
Comprehensive Cancer
Center at Johns Hopkins
Todd L. Demmy, MD
Roswell Park Cancer Institute
Ritsuko Komaki, MD
The University of Texas
MD Anderson Cancer Center
Lee M. Krug, MD
Memorial Sloan-Kettering
Cancer Center
Ramaswamy Govindan, MD
Siteman Cancer Center
at Barnes-Jewish Hospital
and Washington University
School of Medicine
Wallace Akerley, MD
Huntsman Cancer Institute
at the University of Utah
Frederic W. Grannis, Jr., MD
City of Hope Comprehensive
Cancer Center
Mark G. Kris, MD
Memorial Sloan-Kettering
Cancer Center
Hossein Borghaei, DO, MS
Fox Chase Cancer Center
Stefan C. Grant, MD, JD
University of Alabama at
Birmingham Comprehensive
Cancer Center
Rudy P. Lackner, MD
UNMC Eppley Cancer Center at
The Nebraska Medical Center
Andrew C. Chang, MD
University of Michigan
Comprehensive Cancer Center
Richard T. Cheney, MD
Roswell Park Cancer Institute
Lucian R. Chirieac, MD
Dana-Farber/Brigham and
Women’s Cancer Center
Thomas A. D’Amico, MD
Duke Cancer Institute
Leora Horn, MD, MSc, FRCPC
Vanderbilt-Ingram Cancer Center
Thierry M. Jahan, MD
UCSF Helen Diller Family
Comprehensive Cancer Center
Melissa Johnson, MD
Robert H. Lurie Comprehensive
Cancer Center of Northwestern
University
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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Feng-Ming (Spring) Kong, MD, PhD,
MPH
University of Michigan
Comprehensive Cancer Center
Inga T. Lennes, MD
Massachusetts General Hospital
Cancer Center
Billy W. Loo, Jr., MD, PhD
Stanford Cancer Institute
Renato Martins, MD, MPH
Fred Hutchinson Cancer
Research Center/Seattle
Cancer Care Alliance
NCCN Panel Members for Malignant Pleural Mesothelioma
Gregory A. Otterson, MD
The Ohio State University
Comprehensive Cancer Center –
James Cancer Hospital and
Solove Research Institute
Jyoti D. Patel, MD
Robert H. Lurie Comprehensive
Cancer Center of Northwestern
University
Mary C. Pinder-Schenck, MD
Moffitt Cancer Center
Katherine M. Pisters, MD
The University of Texas
MD Anderson Cancer Center
Karen Reckamp, MD, MS
City of Hope Comprehensive
Cancer Center
Gregory J. Riely, MD, PhD
Memorial Sloan-Kettering
Cancer Center
Theresa A. Shapiro, MD, PhD
The Sidney Kimmel
Comprehensive Cancer
Center at Johns Hopkins
Scott J. Swanson, MD
Dana-Farber/Brigham and
Women’s Cancer Center
Kurt Tauer, MD
University of Tennessee
Cancer Institute
Douglas E. Wood, MD
Fred Hutchinson Cancer
Research Center/Seattle
Cancer Care Alliance
Stephen C. Yang, MD
The Sidney Kimmel
Comprehensive Cancer
Center at Johns Hopkins
Eric Rohren, MD, PhD
The University of Texas
MD Anderson Cancer Center
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
53
NCCN Member Institutions
City of Hope Comprehensive
Cancer Center
Los Angeles, California
800.826.4673
cityofhope.org
Dana-Farber/Brigham and
Women’s Cancer Center
Massachusetts General Hospital
Cancer Center
Boston, Massachusetts
800.320.0022
dfbwcc.org
massgeneral.org/cancer
Duke Cancer Institute
Durham, North Carolina
888.275.3853
http://www.dukecancerinstitute.org
Fox Chase Cancer Center
Philadelphia, Pennsylvania
888.369.2427
foxchase.org
Huntsman Cancer Institute
at the University of Utah
Salt Lake City, Utah
877.585.0303
huntsmancancer.org
Fred Hutchinson Cancer
Research Center/
Seattle Cancer Care Alliance
Seattle, Washington
206.288.7222 • seattlecca.org
206.667.5000 • fhcrc.org
The Sidney Kimmel
Comprehensive Cancer
Center at Johns Hopkins
Baltimore, Maryland
410.955.8964
hopkinskimmelcancercenter.org
Robert H. Lurie Comprehensive
Cancer Center of Northwestern
University
Chicago, Illinois
866.587.4322
cancer.northwestern.edu
Memorial Sloan-Kettering
Cancer Center
New York, New York
800.525.2225
mskcc.org
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
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54
Moffitt Cancer Center
Tampa, Florida
800.456.3434
moffitt.org
The Ohio State University
Comprehensive Cancer Center James Cancer Hospital and
Solove Research Institute
Columbus, Ohio
800.293.5066
cancer.osu.edu
Roswell Park Cancer Institute
Buffalo, New York
877.275.7724
roswellpark.org
Siteman Cancer Center
at Barnes-Jewish Hospital
and Washington University
School of Medicine
St. Louis, Missouri
800.600.3606
siteman.wustl.edu
NCCN Member Institutions
St. Jude Children’s
Research Hospital/
University of Tennessee
Cancer Institute
Memphis, Tennessee
888.226.4343 • stjude.org
877.988.3627 • utcancer.org
Stanford Cancer Institute
Stanford, California
877.668.7535
cancer.stanfordhospital.com
University of Alabama at
Birmingham Comprehensive
Cancer Center
Birmingham, Alabama
800.822.0933
ccc.uab.edu
UCSF Helen Diller Family
Comprehensive Cancer Center
San Francisco, California
800.888.8664
cancer.ucsf.edu
University of Michigan
Comprehensive Cancer Center
Ann Arbor, Michigan
800.865.1125
mcancer.org
UNMC Eppley Cancer Center at
The Nebraska Medical Center
Omaha, Nebraska
800.999.5465
unmc.edu/cancercenter
The University of Texas
MD Anderson Cancer Center
Houston, Texas
877.632.6789
mdanderson.org
Vanderbilt-Ingram Cancer Center
Nashville, Tennessee
800.811.8480
vicc.org
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
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Index
Anxiety 30, 33
Asbestos 9, 11, 36
Biopsy 12–14, 17
Cancer cells 7, 9, 12, 13, 16, 20, 22, 24, 25, 27
Cancer spread 7
Cancer staging 16, 19, 20, 39
Chemotherapy 22, 24, 27
Chest wall 6, 17, 19, 29
Computed tomography 11–14, 16, 17, 22, 24–26, 29
Depression 30
Gene changes 7, 9
Lymph nodes 7, 16, 17, 19, 23
Magnetic resonance imaging 16, 17
Mediastinoscopy 16, 17
Member institutions 54, 55
Mesothelium 4, 6, 7, 14
National Comprehensive
Cancer Network 2, 51
Panel members 52, 53
Pleura 6, 9, 11, 13, 17, 19, 23, 29
Pleural biopsy 11, 13, 14, 17
Pleural cavity 6, 11, 12, 29
Pleural mesothelioma 7
Positron emission tomography 16, 29
Primary treatment 23
NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
Radiation therapy 23–26, 29
Risk factors 9
Serum mesothelin-related peptide 11, 14
Supportive care 29, 30
Surgery 21–24
Extrapleural pneumonectomy 23, 24
Pleurectomy/decortication 23, 24
Symptoms 11, 29, 30, 33, 34
Tests 11–17, 21, 22
Diagnosis 11–14
Treatment planning 16, 17, 21, 33
Thoracentesis 11, 12
Treatment 21–27
Adjuvant 24, 25
Neoadjuvant 22
Primary 23
Treatment plan 32–34
Thoracoscopic biopsy 13, 14, 17
56
Notes
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NCCN Guidelines for Patients®: Malignant Pleural Mesothelioma
Version 1.2012
58
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