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Skin Lymphoma

​This information should not be used for self-diagnosis or in place of a qualified physician's care.

​Revised Feb 2016

The basics
  • Guidelines for treating this cancer have been developed by the Lymphoma Tumour Group. These types of cancers are also referred to as lymphoid cancers.
  • For health professional information on treating this cancer, please see our Cancer Management Guidelines: Skin Lymphoma and Cutaneous Lymphoma.
  • Some names of skin lymphomas are cutaneous T-cell lymphomas (CTCL), mycosis fungoides and Sézary syndrome.
  • This rare type of lymphoma behaves very differently from other lymphomas. The lymphocytes (a kind of white blood cell) become cancerous and affect the skin.
  • Mycosis fungoides has nothing to do with a fungus infection.
  • This cancer almost always starts on the skin. It can be present on the skin for years before spreading to other parts of the body.
  • Skin lymphomas are difficult to diagnose because symptoms are similar to other less serious skin diseases. A pathologist can confirm the diagnosis only when the cancer is advanced and there are enough lymphoma cells concentrated on the skin.
  • For this reason, it is not usually diagnosed in people under the age of 40, even though symptoms may have been noted for years and the patient may even have been under the care of a dermatologist (skin specialist).
  • Patients with skin lymphoma at stage I or II usually have a very long life expectancy and some will never relapse after treatment.

What causes it and who gets it?

Listed below are some of the known risk factors for this cancer. Not all of the risk factors below may cause this cancer, but they may be contributing factors.
 
  • No specific causes of skin lymphoma are known. Genetic damage to a person's cells, or immune system suppression (such as caused by AIDS), may be risk factors.
  • Certain specific skin conditions (plaque parapsoriasis, follicular mucinosis) can develop into skin lymphoma.
  • Statistics
    • Skin lymphomas (T-cell or B-cell, mycosis fungoides, or Sezary syndrome) are very rare.
    • In the statistics below, only 5% of those diagnosed with Non-Hodgkin lymphoma will have skin lymphoma. In BC, that might be about 45 people each year.
      • B.C. statistics for these cancers are included with Non-Hodgkin Lymphomas.
      • Canadian statistics are also group this cancer in with other Non-Hodgkin Lymphomas. Skin lymphomas are only a very small number of these cases.

Can I help to prevent it?

There are no definite prevention recommendations for skin lymphomas.

Clinical trials and programs such as the Tumour Tissue Repository hope to learn more about the causes, and prevention, of skin lymphoma in the future.

Screening for this cancer

No effective screening program exists for this cancer yet.

A few skin conditions (plaque parapsoriasis, follicular mucinosis) can develop into skin lymphoma. People with these conditions may be checked often, by their doctor, for changes in their disease.

Signs and symptoms

  • A chronic rash, often itchy, may appear anywhere on the surface of the body.
  • In early stages it appears as either thin red or scaly patches on the skin, or thicker areas of scaly skin (called plaques) that often look like eczema or psoriasis.
Diagnosis & staging

Diagnosis

These are tests that may be used to diagnose this type of cancer.
  
  • A careful physical exam is done to look for signs of skin lymphoma, such as lumps, skin lesions, and any other symptoms.
  • A biopsy is the only way to diagnose skin lymphomas such as mycosis fungoides.
  • For a skin biopsy, affected skin cells are removed by minor surgery in the doctor's office and the sample will be examined under a microscope.
  • Other tests to check the extent of the disease may include a blood test, or a CT scan of the abdomen and pelvis.
  • If a lymph node biopsy is needed, some cells may be removed with a needle or in surgery, and will be examined under a microscope.
For more information on tests used to diagnose cancer, see our Recommended Websites, Diagnostic Tests section.
  

Types and stages

Types
  • Skin lymphomas are often given a name based on the appearance of the lymphoma cells under a microscope.
  • Mycosis fungoides is the most common cutaneous T-cell lymphoma (CTCL), which starts in cells called T-lymphocytes (white blood cells).
  • Sézary syndrome is like mycosis fungoides, but the lymphoma cells are also found in the blood or the lymph nodes.
  • Different kinds of T-cells can become cancerous. Other kinds of T-cell lymphoma which can affect the skin are:
    • primary cutaneous anaplastic large cell
    • lymphomatoid papulosis
    • subcutaneous panniculitis-like T-cell lymphoma
    • primary cutaneous peripheral T-cell lymphoma, a rare group including epidermotropic CD8+ cytotoxic, gamma/delta, or CD4+ small/medium sized pleomorphic.
  • Cutaneous B-cell lymphoma starts with cells called B-lymphocytes, a white blood cell which makes antibodies. Some B-cell lymphomas are:
    • primary cutaneous marginal-zone B-cell lymphoma
    • primary cutaneous follicle-centre lymphoma
    • primary cutaneous diffuse large B-cell lymphoma, leg type or non-leg.
Stages
Staging describes the extent of a cancer. The TNM classification system is used as the standard around the world. In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.

T describes the site and size of the main tumour (primary)

N describes involvement of lymph nodes; NP describes peripheral nodes; NV is visceral nodes

M relates to whether the cancer has spread (presence or absence of distant metastases)

B (for skin lymphoma) describes whether lymphoma cells are circulating in the blood

Patients with biopsy-confirmed CTCL (cutaneous t-cell lymphoma) are staged according to the extent of their skin disease and treatment is determined by their stage. The staging system used is as follows:

  • T0
    No skin involvement
  • T1
    Patches or plaques, covering <10% of the body surface
  • T2
    Same as above but covering >10% of the body surface
  • T3
    Tumors with or without patches or plaques
  • T4
    Generalized erythema
  • NP0
    No abnormal peripheral lymph nodes
  • NP1-
    Abnormal peripheral lymph nodes, biopsy negative
    (i.e., dermatopathic lymphadenopathy)
  • NP1+
    Abnormal peripheral lymph nodes, biopsy positive
  • NV0
    No abnormal visceral lymph nodes
  • NV1
    Abnormal visceral lymph nodes (no information regarding biopsy)
  • NV1-
    Abnormal visceral lymph nodes, biopsy negative
  • NV1+
    Abnormal visceral lymph nodes, biopsy positive
  • M0
    No visceral organ involvement
  • M1
    Visceral organ involvement (on basis of histology)
  • B0
    <5% circulating Sézary cells and <250 Sézary cells per cc
  • B1
    >5% circulating Sézary cells, or >250 Sézary cells per cc
To determine the extent of the disease the following procedures may be requested:
  • Immunological laboratory tests on blood
  • Chest X-ray
  • CT scan of the abdomen
  • Examination of the blood for lymphoma cells and other blood tests
Treatment

Treatment

Cancer therapies can be highly individualized – your treatment may differ from what is described below. 

External treatment of the skin
  • Treatment of stages I and II may be a chemotherapy agent applied to the skin. For skin lymphomas, the main chemotherapy drug used on the skin is Nitrogen Mustard (Mechlorethamine) ointment.
  • Another treatment option for stages I or II can be ultraviolet light therapy (PUVA), using a drug (Psoralen) to make the lymphoma cells sensitive to the light.
  • External treatments for lymphomas of the skin are not the same as treatments used on other skin cancers.
Surgery
  • Surgery is not used to treat skin lymphomas, although a surgical biopsy (sample) of the area might be used to understand the stage of it.
Radiation Therapy
  • Radiation therapy of the involved part of the skin is usually very effective.
  • Radiation therapy to the whole skin surface may be used if external skin treatments are not effective.
Chemotherapy
  • Chemotherapy may be recommended if the skin lymphoma has spread. Drugs used for these lymphomas in the skin are not the same as drugs used for other skin cancers.
  • Some chemotherapy drugs are given as pills, to be taken by mouth. Others are given intravenously, through a needle in a vein (IV). Useful drugs include Acitretin, Chlorambucil, or Gemcitabine.
  • Radiation therapy to affected areas of the skin may be used at the same time.

Follow-up after treatment

  • You will be returned to the care of your family doctor or specialist for regular follow-up. If you do not have a family physician, please discuss this with your BC Cancer Agency oncologist or nurse.
  • Follow-up testing is based on your type of cancer and your individual circumstances.
  • Life after Cancer focuses on the issues that cancer survivors can face.
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SOURCE: Skin Lymphoma ( )
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