• 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

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 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.