Download Mycosis Fungoides

RUNNING HEAD: Mycosis Fungoides
MYCOSIS FUNGOIDES
Cutaneous T-Cell Lymphoma (CTCL)
SAMANTHA CROWELL
GRAND VALLEY STATE UNIVERSITY
0
Mycosis Fungoides
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Presenting Signs & Symptoms of Patient
Chief Complaint
This patient is a white 52 year old female with the diagnosis of Mycosis fungoides,
cutaneous T-cell lymphoma with a symptomatic lesion on her forehead and neck. She had
noticed in the spring of 2015 that her skin nodules had increased in size and she developed new
lesions on her forehead and left eyebrow. She then had a PCP performed biopsy that showed
inflammation. After having a biopsy on the in early October of 2015, her pathology was
consistent with atypical dermal and folliculotropic CTCL with TCR gene arrangement. On a
repeat biopsy 6 days later, it documented to show mycosis fungoides with negative TCR gene
arrangement, stage IVB. She had multiple erythematous tumors measuring approximately 1-3 cm
located across her right forehead for combined length of 10 cm. The lateral left eyelid was
erythematous and enlarged. On her left supraclavicular fossa, there were 2 raised lesions less
than 1 cm wide. Multiple lesions were present on her back and trunk including a 4 cm plaque on
her mid-back. Erythematous patches were present on both forearms and legs. She had already
started UV therapy and had seen improvement on most of her lesions. She presented to radiation
oncology to discuss having radiation treatment.
Medical History
The patient has a history with previous cancer. She was previously treated for a diagnosis
of T1cpN0, grade 3, invasive ductal carcinoma of the left breast in 2009. She underwent a
lumpectomy and sentinel node biopsy. Her tumor was ER, PR and HER-2/neu negative. She was
treated with 5040 cGy to the left breast, followed by a boost to the tumor bed for a total dose of
6040 cGy. She also had chemotherapy during this time. These treatments were completed by
December of 2009. She has had UV therapy for her current condition, but was seeking additional
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treatment. This patient also has a long history with eczema which has been treated with
Clobetasol cream. She began menstruating at the age of 13. This patient has had 1 pregnancy
along with 1 birth.
Family History
Mother has experienced head and neck cancer. Sister has experienced cervical cancer.
Maternal grandmother is deceased having experienced uterine cancer. Maternal grandfather is
deceased having experienced bone cancer.
Social History
Patient is currently an every day smoker. She smokes a half pack per day and has done so
for 30 years (15.0 pack years). Patient does not have a history of drinking.
Epidemiology
Cutaneous lymphomas signify about 3.9% of all non-Hodgkin lymphomas. Mycosis
fungoides accounts for most of these incidences. The occurrence of cutaneous T-cell lymphoma
in the United States has gone up since 1973, going from a yearly incidence rate of 6.4 events per
million to 9.6 events per million people. Mycosis fungoides usually occurs in older people
having a median age at time of diagnosis of 55-60 years. Although, the disease can be found in
some young aged people as well. The incidence is also skewed between sexes with a male to
female ratio of 2:1. About 70% of patients with mycosis fungoides in the US are white in race.
Patients who are Black, Hispanic, and Asian in race account for approximately 14%, 9%, and 7%
of the mycosis fungoides cases in the US. T-cell lymphoma in adults is common in areas with a
high occurrence of HTLV-1 infection. These areas include southwest Japan, the Caribbean
islands, South America, and parts of central Africa. Having mycosis fungoides significantly
increases a patient’s risk of developing a second lymphoma, especially risks of the cutaneous T-
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cell lymphoma subtype lymphomatoid papulosis and nonhematologic malignancies. The most
common locations for mycosis fungoides to develop are the upper thighs and groin, breasts,
armpits, and the crook of the elbow. (Jewad, S., et al., 2014 & Medscape, 2016)
Etiology
The primary sources for cutaneous T-cell lymphoma mycosis fungoides have not been
discovered yet. However, there is evidence that mycosis fungoides could be in relation to a
chronic inflammatory skin disease which is directed by T-cells. (Medscape, 2016) In addition,
mycosis fungoides has been thought to stem from a chronic antigenic stimulation. This condition
leads to an uncontrolled clonal development which creates a buildup of lymphatic T-cells within
the skin. Researchers have found that in early mycosis fungoides tumors there an increased
numbers of dendritic cells present. There is also evidence supporting the idea that neoplastic Tcells can release APC ligands. This would propose the idea of T-cell buildups being caused by
some sort of self-stimulation. If a patient has HIV or has had a type of immunosuppression done
to their system prior to an organ transplant this could increase their risk of developing a
cutaneous T-cell lymphoma. Other occupational exposures have been researched, however, there
is not enough evidence to support their involvement with mycosis fungoides either way. (Jawed,
S., et al., 2014)
Comparison of Patient to the Typical
This patient seems to be somewhat typical for this disease. This patient is white in race
which is very typical for this disease. She is a middle aged which is most common, however, she
is a female and this disease is less common in females than it is in males. The patient also was
diagnosed with the disease when it was already in a late stage. This is atypical because around
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70% of patients at the time of diagnosis are still in the early stage of disease. (Jawed, S., et al.,
2014)
Patient Work-Up Information
Date
Procedure
Results
Spring of
2015
Chief Complaint
Patient noted that her skin nodules had increased in
size. She also noticed new lesions on forehead and
left eyebrow.
October 6,
2015
Biopsy at Dermatologist
Pathology was consistent with atypical dermal and
folliculotropic CTCL with TCR gene arrangement.
October 12,
2015
Repeat Biopsy
Pathology showed mycosis fungoides with negative
TCR gene arrangement.
November
2015
Dermatologist in
Another Clinic
Patient was seen by an experienced dermatologist
and discussed at tumor board.
December
2015
Radiation Oncologist in
Another Clinic
Physician recommended treatment to lesions of her
forehead, left eyelid, and left supraclavicular
regions.
December
2015
Patient Began UV
Therapy for Her Lesions
Patient noticed improvement in most of her lesions.
December
2015
Patient Examined by
New Radiation
Oncologist
Discussed additional treatment options and decided
on a course of palliative radiation therapy.
January 19,
2016
Simulation for
Radiation Therapy
N/A
Anatomy Discussion
The lymphatic system is made up of a clear fluid called lymph, lymphoid tissue, and
lymph vessels. The lymphoid tissue is composed from multiple different immune system cells
that help the entire body fight off infections together. The majority of these cells are a type of
white blood cell called lymphocytes. Lymphocytes are housed within the lymph nodes and other
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lymphoid tissues such as the spleen, skin, bone marrow, thymus, adenoids, tonsils, digestive tract
and other organs. There are two main types of lymphocytes called B-cells and T-cells, as shown
in Figure 1. The B-cells make proteins called antibodies which help to protect the body against
bacteria and viruses by attaching themselves and marking them for destruction. T-cells also
protect the body against germs, but also can release material called cytokines which draw in
other white blood cells to digest the diseased cells. The T-cells can also slow down or speed up
the actions of other immune system cells.
Lymphoma is a neoplasm that starts in the body’s immune system from these
lymphocytes. There are two main types of lymphomas which are Hodgkin’s lymphoma and nonHodgkin’s lymphoma. If a lymphoma originates on the skin, it is a skin lymphoma. Skin
lymphomas are a non-Hodgkin’s lymphoma. Lymphoma cells can develop from both B-cells and
T-cells, however, they are more commonly arisen from T-cells when referencing to the skin.
(American Cancer Society) Mycosis fungoides is a neoplasia of a malignant lymphatic T-cell.
(Boulos, S., et al., 2014) This type of neoplasia has been categorized as a cutaneous T-cell
lymphoma because of their initial presentation as skin involvement. In Figure 2, it is shown how
mycosis fungoides originate from lymphatic T-cells and can make their involvement within the
skin. (National Cancer Institute)
Regional Lymphatic Drainage
The involvement of the lymph system is huge with cutaneous T-cell lymphoma because
the patient’s cancer originated from lymph cells. This patient has lesions on her forehead, eyelid,
supraclavicular fossa, back, trunk, her forearms and legs. This means that the cancer has already
spread throughout her entire lymphatic system. Figure 3 presents the lymphatic system is huge
and covers the entire body.
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Anatomy & Lymphatic Graphics
FIGURE 1.
Mycosis Fungoides and the Sézary Syndrome Treatment. (n.d.). National Cancer Institute.
Retrieved February 15, 2016, from
http://www.cancer.gov/types/lymphoma/patient/mycosis-fungoides-treatment-pdq
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FIGURE 2.
Compton, C. (2012). Primary Cutaneous Lymphomas. In AJCC Cancer Staging Atlas: A
Companion to the Seventh Editions of the AJCC Cancer Staging Manual and Handbook
(2nd ed., pp. 619-626). New York, NY: Springer. Retrieved February 18, 2016, from
GVSU Online Library.
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FIGURE 3.
Hilmers Studios, Medical Illustrations. (n.d.). Retrieved February 20, 2016, from
http://www.hilmersstudios.com/medical#anchor_lymphatic-system_
Pathology
The histologic diagnosis of mycosis fungoides is not easy to figure out when in the earlier
stages of development. Even pathologists with a lot of experience usually need to view several
biopsies before making a diagnosis. The National Cancer Institute states “A definitive diagnosis
from a skin biopsy requires the presence of MF/SS cells (convoluted lymphocytes), a band-like
upper dermal infiltrate, and epidermal infiltrations with Pautrier abscesses (collections of
neoplastic lymphocytes)”. All of the information needed to make a proper diagnosis may not be
available in a single biopsy alone. It is important to note that even though skin cells are highly
involved with this cancer, the actual skin cells themselves are not cancerous. (National Cancer
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Institute) Sézary Syndrome is very similar to Mycosis fungoides and is often thought to be an
advanced stage of it. It is an aggressive and cutaneous T-cell lymphoma, which is identified by
“circulating atypical T cells (Sézary cells), diffuse erythema (erythroderma), and severely
disabling pruritus with or without lymphadenopathy” (Jawed, S., et al., 2014). It is often thought
that Sézary Syndrome is not associated with mycosis fungoides, however, sometimes it is
presented after a patient had mycosis fungoides. The debate continues on whether they are
related or not. (Jawed, S., et al., 2014)
Staging
As a standard way of classifying the degree of a patient’s cancer, the American Joint
Committee on Cancer (AJCC) created the TNM staging system. This staging system can be
applied to staging of Mycosis fungoides and Sézary Syndrome. TNM usually uses four key
concepts which include the T which refers to the location, size, how far it has spread within the
skin and nearby tissues; the N which describes how much the cancer has spread to nearby lymph
nodes; the M which indicates whether the cancer has metastasized. With staging these T-cell
lymphomas, the TNM system also added the category of peripheral blood involvement, B. Each
of these four categories have their own staging guide. When all four of them are determined, they
are combined in a process called stage grouping to assign an overall stage for the tumor. Having
accurate staging is vital to allow the patient to have the correct treatment and prognosis.
The following tables were created to show the staging process of Mycosis fungoides and
Sézary Syndrome and all of the different components involved in it. All of the staging
information in the following tables was taken from the AJCC handbook.
Primary Tumor (T)
TNM
Categories
Description
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Limited patches, *papules, and/or plaques **covering less than 10% of the skin
surface.
T1
*For skin, patch indicates any size skin lesion without significant elevation or induration.
Presence/absence of hypo- or hyperpigmentation, scale, crusting, and/or poikiloderma should
be noted.
**For skin, plaque indicates any size skin lesion that is elevated or indurated. Presence or
absence of scale, crusting, and/or poikiloderma should be noted. Histologic features such as
folliculotropism or large-cell transformation (>25% large cells), CD30+ or CD30–, and clinical
features such as ulceration are important to document
T1a
Patch only
T1b
Plaque ± patch
T2
Patches, papules or plaques covering 10% or more of the skin surface.
T2a
Patch only
T2b
Plaque ± patch
One or more tumors ***(1≥-cm diameter)
T3
T4
***For skin, tumor indicates at least one 1-cm diameter solid or nodular lesion
with evidence of depth and/or vertical growth. Note total number of lesions,
total volume of lesions, largest size lesion, and region of body involved. Also
note if histologic evidence of large-cell transformation has occurred.
Phenotyping for CD30 is encouraged
Confluence of erythema covering 80% or more of body surface area
Lymph Nodes Involvement (N)
TNM
Categories
Description
Clinically abnormal peripheral lymph nodes****; no histologic confirmation
NX
N0
****For node, abnormal peripheral lymph node(s) indicates any palpable
peripheral node that on physical examination is firm, irregular, clustered,
fixed or 1.5cm or larger in diameter. Node groups examined on physical
examination include cervical, supraclavicular, epitrochlear, axillary, and
inguinal. Central nodes, which are not generally amenable to pathologic
assessment, are not currently considered in the nodal classification unless
used to establish N3 histopathologically
No clinically abnormal peripheral lymph nodes; biopsy not required
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Clinically abnormal peripheral lymph nodes; histopathology
N1
Dutch grade 1 or NCI LN0-2
Clone negative*****
N1a
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Clone positive*****
N1b
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Clinically abnormal peripheral lymph nodes; histopathology
N2
Dutch grade 2 or NCI LN3
Clone negative*****
N2a
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Clone positive*****
N2b
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Clinically abnormal peripheral lymph nodes; histopathology
N3
Dutch grades 3–4 or NCI LN4; clone positive or negative
Visceral Metastasis (M)
TNM
Categories
Description
M0
No visceral organ involvement
M1
Visceral involvement (must have pathology confirmation^ and organ
involved should be specified)
^ For viscera, spleen and liver may be diagnosed by imaging criteria.
Peripheral Blood Involvement
TNM
Categories
B0
Description
Absence of significant blood involvement: 5% or less of peripheral blood
lymphocytes are atypical (Sézary) cells^^
^^ For blood, Sézary cells are defined as lymphocytes with hyperconvoluted cerebriform
nuclei. If Sézary cells are not able to be used to determine tumor burden for B2, then one of
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the following modified ISCL criteria along with a positive clonal rearrangement of the TCR
may be used instead: (1) expanded CD4+ or CD3+ cells with CD4/CD8 ratio of 10 or more,
(2) expanded CD4+ cells with abnormal immunophenotype including loss of CD7 or CD26.
Clone negative*****
B0a
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Clone positive*****
B0b
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
B1
Low blood tumor burden: more than 5% of peripheral blood lymphocytes are
atypical (Sézary) cells but does not meet the criteria of B2
Clone negative*****
B1a
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Clone positive*****
B1b
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
B2
High blood tumor burden: 1000/ mL Sézary cells^^ or more with positive
clone
*****A T-cell clone is defined by PCR or Southern blot analysis of the T-cell receptor
gene.
Anatomic Stage/Prognostic Groups
TNM Stages
T
N
M
Peripheral
Blood
Involvement
Stage IA
1
0
0
0, 1
Stage IB
2
0
0
0, 1
Stage IIA
1, 2
1, 2
0
0, 1
Stage IIB
3
0-2
0
0, 1
Stage III
4
0-2
0
0, 1
Stage IIIA
4
0-2
0
0
Stage IIIB
4
0-2
0
1
Stage IVA1
1-4
0-2
0
2
Stage IVA2
1-4
3
0
0-2
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Stage IVB
1-4
0-3
1
0-2
Compton, C. (2012). Primary Cutaneous Lymphomas. In AJCC Cancer Staging Atlas: A
Companion to the Seventh Editions of the AJCC Cancer Staging Manual and Handbook
(2nd ed., pp. 619-626). New York, NY: Springer. Retrieved February 18, 2016, from
GVSU Online Library.
This patient has Stage IVB cutaneous T-cell lymphoma mycosis fungoides. From the
tables above, it is shown that this means the patient could have any type of primary tumor
presentation, lymph node involvement, or peripheral blood involvement. The specifics were not
documented in her charts. However, this patient does have visceral metastasis. She has the most
progressive stage of mycosis fungoides.
Grading
There currently is not a grading system for Mycosis fungoides. However, there is
research supporting that use of a grading system would improve the diagnosis of mycosis
fungoides substantially. A study published in 2001 tested a new system for interpreting and
reporting biopsies suspicious for MF. The process was centered on a grading system revealing
the level of diagnostic certainty from the pathologist. The researchers found that the agreement
percentage on histological diagnosis of mycosis fibrosis among the pathologists improved from
48% to 76% when using the grading system. Additional research is needed in this area for future
improvement. (Guitart, J. et al., 2001)
Patients Pathology, Stage & Grade
She then had a PCP performed biopsy that showed inflammation. After having a biopsy
on the in early October of 2015, her pathology was consistent with atypical dermal and
Mycosis Fungoides
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folliculotropic CTCL with TCR gene arrangement. On a repeat biopsy 6 days later, it
documented to show mycosis fungoides with negative TCR gene arrangement. Her formal
diagnosis was noted as cutaneous T-cell lymphoma, mycosis fungoides, stage IVB, with positive
lymph nodes in multiple sites. Grade is not assessed for this condition.
Radiation Therapy Treatment Plan & Rx for Patient
The patient was prescribed to 2400 cGy to the right forehead, temple, and supraclavicular
neck. This was to be delivered in 12 fractions of 200 cGy each with electrons. The doctor
decided not to treat the left eyelid because it had decreased and due to its location and proximity
to the eye.
Radiation Therapy Treatment Information & Patient Set-Up
Simulation for the patient took place on January 19, 2016. She was to lie head first on the
table in a supine position with an IMRT headboard and open faced IMRT mask. Under her head
was a size ‘B’ headrest and her hands were to be at her sides to keep her arms out of the field.
She also had a large knee sponge under her legs to keep her comfortable and in place. There
were marks drawn on the mask from the doctor to create a matching electron cutout light field.
For treatment she was to be clinically set-up matching the light field to the outlines on the mask.
Her SSD needed to be set to 100cm on her forehead and 110cm for her supraclavicular field.
This patient was prescribed to 2 different electron fields. A 1cm bolus is required for each field
daily.
Radiation Therapy Treatment Type & Delivery
Field
Name
Gantry
Angle
Collimator
Angle
Couch
Angle
Planned
SSD
Forehead
303.0
35
335
100.0
cm
Planned
Energy
MU
Cone
Size
Bolus
(cm)
198 MU
15X15
1.0
9 MeV
Mycosis Fungoides
Lt Sclv
60.0
15
75
75
110.0
cm
246 MU
9 MeV
15X15
1.0
Radiation Therapy Complications, Side Effects & Treatment
This patient had a few complications from her radiation therapy treatments. After 10 Gy
in 5 fractions, she had complained of fatigue. She denied having lack of appetite and fever. The
patient also complained of dry skin. The areas to her right forehead, left clavicle, and back were
red but the patient seemed to think they were improving because they were getting smaller. The
physician stated that at this point her lesions were flattening out. She also complained of having
migraines. When her treatment reached 18 Gy in 9 fractions, the patient had very similar
complaints and complications. The only addition is the migraines have been more frequent and
she complained about a pressure in her head. Once the patient completed her treatment of 24 Gy
in 12 fractions, her lesions were not gone but looked more flat and smooth. She has been
instructed to follow up in a month to see the progress.
Adjuvant Therapies, Complications, Side Effects & Treatment
Six types of standard treatment are used: Photodynamic therapy, Radiation therapy,
Chemotherapy, Other drug therapy, Biologic therapy & Targeted therapy. Photodynamic therapy
uses a combination of drug and a laser light that work together to kill cancer cells with little
damage to the surrounding healthy tissue. The drug is specially formulated to accumulate within
tumor cells and remain inactive until exposed to light. It is injected into a vein and the laser light
is directed onto the affected area which activates the drug to kill the tumor cells. (Humme, D.,
Nast, A., Erdmann, R., Vandersee, S., & Beyer, M., 2014)
Mycosis fungoides can also be treated with a type of radiation therapy called total skin
electron beam radiation therapy. This treatment type uses rays of electrons on the skin surface of
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the entire body. Electrons treat superficially, so, the normal underlying tissues and organs will be
unaffected. Another type of radiation that can be used is ultraviolet B radiation therapy. This
uses a special light that directs UVB radiation over the skin. (National Cancer Center)
Chemotherapy uses drugs to prevent cancer cells from growing, either by killing them or
by preventing them from dividing. When the drugs are taken orally or injected into the body
through either a vein or into a muscle, the drugs are able to reach the whole body through the
bloodstream. This is called a systemic treatment because it treats the entire system of a body.
This is a good treatment option for cancers that have started to metastasize because the distant
cancer cells won’t be missed. Chemotherapy can also be given topically through the use of a
cream, lotion, or ointment on the skin. For example, topical corticosteroids can be applied to
relieve painful symptoms of red, swollen, and inflamed skin. They come in many forms of
application. (Panasiti, V, et al., 2006) The type of chemotherapy chosen for a patient depends on
their disease type and stage that needs to be treated. (National Cancer Center)
Another treatment called biologic therapy uses different substances to activate a patient’s
own immune system to fight off their cancer. These substances are made either in the body or
within a laboratory. The goal of them is to enhance, instruct, or repair the natural defenses of the
patient’s body against their cancer. Mycosis fungoides can be treated with a substance called
Interferon as a type of biologic therapy. Interferon interferes with the reproduction of neoplastic
cells and can slow the already existing tumor from growing. A similar treatment called targeted
therapy uses certain drugs or substances to search for and destroy specific types of cancer cells.
The drugs are designed to not harm normal cells. Monoclonal antibody therapy is a specific
targeted therapy used to treat mycosis fungoides. This cuts off the nutritional supply to the tumor
and stops it from growing and in most cases kills the tumor. (National Cancer Society)
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The treatment that is prescribed and delivered to the patient will depends on the type and stage of
the cancer being treated. Treatments will vary by patient.
Other Therapies & Complications the Patient Received
This patient also received UV therapy for her disease. No complications or details on
treatment were noted. This was performed in a different cancer research facility and cancer
treatment center.
Critical Structures & Dose Tolerances
When treating patients with mycosis fungoides the main critical structure that needs to be
focused on is the skin. This is because the lesions are involved with the skin in multiple locations
in most patients. If the skin dose goes past the tolerance, then it could result in acute or chronic
dermatitis. For this particular patient, her disease was on her head and neck region. This means
that there are some other main critical structures that need to be watched as well. The lenses of
the eyes have a really low tolerance dose and she is being treated on her face, so, these need to be
monitored closely. If any parts of the eye are damaged including the retina, cornea and lens, then
the injury will be blindness. We are treating a lymphoma, so knowing the tolerance dose of the
lymph nodes and other lymphatics is also important. Passing these tolerance doses would result
in atrophy or sclerosis. Since this patient is being treated with electrons, the treatment dose is
very superficial. This means the internal organs and normal tissues are not at risk for exceeding
their tolerance dose.
Critical Structure
TD 5/5 (cGy)
Lens of eye
500
Cornea of eye
5000
Retina of eye
5500
Skin
5500
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Lymph nodes and lymphatics
5000
Washington, C., & Leaver, D. (2010). Overview of Radiobiology. In Principles and Practice of
Radiation Therapy (3rd ed., pp. 81-82). St. Louis, Mo.: Mosby Elsevier.
Routes of Spread
The routes of spread for mycosis fungoides are through the lymphatic system and the
blood. Since most people are diagnosed at an early stage, they do not make it through all of the
stages. This is in part due to the fact that Mycosis fungoides progresses slowly from stage to
stage. The majority of patients with mycosis fungoides first develop spots on their skin called
patches. These lesions are known to be flat, scaly, pink or red in color, and can be itchy. These
patches are caused by Cancerous T-cells due to them moving from the blood and through the
skin. The skin cells are not actually cancerous, however, the cancerous T-cells can be found
within the patches. The patches do not have a pattern when it comes to presentation. They can
fade and resurface or can remain unchanged over time. If the patches on the patient get worse
they change into plaques which is the next stage of mycosis fungoides. It is a generally a
systematic disease, so it can spread to all parts of the body. It was found “In any stage of mycosis
fungoides, the cancerous T cells can spread to other organs, including the lymph nodes, spleen,
liver, and lungs, although this most commonly occurs in the tumor stage” (Mycosis Fungoides,
2016). Once the tumor spreads into visceral tissue, it is then considered Stage IVB with the worst
prognosis. When the cancer cells spread into an actual tumor they have a possibility of necrotic
ulceration. (Mycosis Fungoides, 2016)
Prognosis & Survival
For patients with mycosis fungoides the prognosis is based greatly on the presentation of
the disease. When there is visceral and peripheral blood involvement and lymphadenopathy, the
Mycosis Fungoides
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likelihood of having cutaneous involvement gets worse. As this gets worse, so does the prognosis
of the patient. The average survival after diagnosis differs between stages. Patients with stage IA
disease have an average survival of 20 years or more. The majority of deaths for patients with
this stage of disease are not in any relation to mycosis fungoides. However, over half of the
patients with stages III-IV disease have death caused by mycosis fungoides. They only have less
than 5 years for an average survival. Since the symptoms of mycosis fungoides often lead to a
misdiagnosis of eczema or other skin irritations, the symptoms can be present for years. It has
been found that the degree of cutaneous involvement, the T scale in the staging system, is
considerably linked with the patient’s prognosis. As involvement increases, there is a decreased
overall survival. In advanced stages of cutaneous involvement some patients have had
progression-free survival. (National Cancer Institute)
Patient’s Prognosis & Survival
Although this patient has seen progress to her lesions, I do not think that her prognosis is
good. Her cancer is at a very progressive stage for this type. The average survival for her stage
IVB is 5 years or less. Also, her physician had planned her treatment to be palliative not curative.
I think she has a 20% chance for a 5-year survival.
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Sources Cited
Articles & Websites
Boulos, S., Vaid, R., Aladily, T. N., Ivan, D. S., Talpur, R., & Duvic, M. (2014). Clinical
presentation, immunopathology, and treatment of juvenile-onset mycosis fungoides: A
case series of 34 patients. Journal of the American Academy of Dermatology, 71(6),
1117-1126. Retrieved February 18, 2016, from ScienceDirect.
Compton, C. (2012). Primary Cutaneous Lymphomas. In AJCC Cancer Staging Atlas: A
Companion to the Seventh Editions of the AJCC Cancer Staging Manual and Handbook
(2nd ed., pp. 619-626). New York, NY: Springer. Retrieved February 18, 2016, from
GVSU Online Library.
Cutaneous T-Cell Lymphoma. (2016, January 15). Medscape. Retrieved February 18, 2016, from
http://emedicine.medscape.com/article/2139720-overview
Guitart, J., Kennedy, J., Ronan, S., Chmiel, J. S., Hsiegh, Y., & Variakojis, D. (2001). Histologic
criteria for the diagnosis of mycosis fungoides: Proposal for a grading system to
standardize pathology reporting. J Cutan Pathol Journal of Cutaneous Pathology, 28(4),
174-183. Retrieved February 20, 2016, from PubMed.
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