General Information About Mycosis Fungoides and the Sézary Syndrome
Stages of Mycosis Fungoides and the Sézary Syndrome
Recurrent Mycosis Fungoides and the Sézary Syndrome
Treatment Option Overview
Treatment Options by Stage

Stage I Mycosis Fungoides and the Sézary Syndrome Stage II Mycosis Fungoides and the Sézary Syndrome Stage III Mycosis Fungoides and the Sézary Syndrome Stage IV Mycosis Fungoides and the Sézary Syndrome

Treatment Options for Recurrent Mycosis Fungoides and the Sézary Syndrome
To Learn More About Mycosis Fungoides and the Sézary Syndrome
Get More Information From NCI
Changes to This Summary (05/06/2008)
About PDQ

General Information About Mycosis Fungoides and the Sézary Syndrome

Key Points for This Section

Mycosis fungoides and the Sézary syndrome are diseases in which lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin. Mycosis fungoides and the Sézary syndrome are types of cutaneous T-cell lymphoma. A possible sign of mycosis fungoides and the Sézary syndrome is a red rash on the skin. Sézary syndrome is an advanced form of mycosis fungoides. Tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and the Sézary syndrome. Certain factors affect prognosis (chance of recovery) and treatment options.

Mycosis fungoides and the Sézary syndrome are diseases in which lymphocytes (a type of white blood cell) become malignant (cancerous) and affect the skin.

Normally, the bone marrow makes blood stem cells (immature cells) that develop into mature blood stem cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell. The myeloid stem cell develops into a red blood cell, white blood cell, or platelet. The lymphoid stem cell develops into a lymphoblast and then into one of three types of lymphocytes (white blood cells):

• B-cell lymphocytes that make antibodies to help fight infection.
• T-cell lymphocytes that help B-lymphocytes make the antibodies that help fight infection.
• Natural killer cells that attack cancer cells and viruses.

Enlarge

Blood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell.

In mycosis fungoides, T-cell lymphocytes become cancerous and affect the skin. In the Sézary syndrome, cancerous T-cell lymphocytes affect the skin and the peripheral blood.

Mycosis fungoides and the Sézary syndrome are types of cutaneous T-cell lymphoma.

This summary describes the two most common types of cutaneous T-cell lymphomas: mycosis fungoides and the Sézary syndrome. For information about other types of skin cancer or non-Hodgkin lymphoma, see the following PDQ summaries:

• Adult Non-Hodgkin Lymphoma Treatment ¹
• Skin Cancer Treatment ²
• Melanoma Treatment ³
• Kaposi Sarcoma Treatment ⁴

A possible sign of mycosis fungoides and the Sézary syndrome is a red rash on the skin.

Mycosis fungoides and the Sézary syndrome may move through the following phases:

• Premycotic phase: A scaly, red rash in areas of the body that usually are not exposed to the sun. This rash does not cause symptoms and may last for months or years. It is hard to diagnose the rash as mycosis fungoides during this phase.
• Patch phase: Thin, reddened, eczema-like rash.
• Plaque phase: Thickened, red patches or reddened skin.
• Tumor phase: Tumors form on the skin. These tumors may develop ulcers and the skin may get infected.

Sézary syndrome is an advanced form of mycosis fungoides.

In the Sézary syndrome, skin all over the body is reddened, itchy, peeling, and painful. There may also be patches, plaques, or tumors on the skin. Cancerous T-cells are found in the blood. Mycosis fungoides does not always progress to the Sézary syndrome.

Tests that examine the skin and blood are used to detect (find) and diagnose mycosis fungoides and the Sézary syndrome.

The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps, the number and type of skin lesions, or anything else that seems unusual. Pictures of the skin and a history of the patient’s health habits and past illnesses and treatments will also be taken.
• Complete blood count with differential: A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells and platelets.
  • The number and type of white blood cells.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.

  Enlarge

  Complete blood count (CBC). Blood is collected by inserting a needle into a vein and allowing the blood to flow into a tube. The blood sample is sent to the laboratory and the red blood cells, white blood cells, and platelets are counted. The CBC is used to test for, diagnose, and monitor many different conditions.

• Peripheral blood smear: A procedure in which a sample of blood is viewed under a microscope to count different circulating blood cells (red blood cells, white blood cells, platelets, etc.) and see whether the cells look normal.
• Biopsy: The removal of cells or tissues so they can be viewed under a microscope to check for signs of cancer. The doctor may remove a growth from the skin, which will be examined by a pathologist. More than one skin biopsy may be needed to diagnose mycosis fungoides.
• Immunophenotyping: A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process may include special staining of the blood cells. It is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.
• Immunogenotyping: A procedure in which a sample of DNA from a skin biopsy is studied to see if the genes for certain kinds of immune system proteins, such as the T-cell receptor or antibody proteins, are arranged in one pattern. Normally T-cell receptor genes and antibody genes are arranged in many different patterns. In mycosis fungoides and the Sézary syndrome, the genes are arranged in a single pattern.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

• The stage of the cancer (the amount of skin affected and whether cancer has spread to the lymph nodes, the blood, or other places in the body).
• The type of lesion (patches, plaques, or tumors).
• The number of cutaneous T-cell lymphocytes in the blood.

Mycosis fungoides and the Sézary syndrome are difficult to cure. Treatment is usually palliative, to relieve symptoms and improve the quality of life. Patients can live many years with this disease.

Stages of Mycosis Fungoides and the Sézary Syndrome

Key Points for This Section

After mycosis fungoides and the Sézary syndrome have been diagnosed, tests are done to find out if cancer cells have spread from the skin to other parts of the body. There are three ways that cancer spreads in the body. The following stages are used for mycosis fungoides and the Sézary syndrome: Stage I Stage II Stage III Stage IV Stages of mycosis fungoides and the Sézary syndrome may also have a B classification.

After mycosis fungoides and the Sézary syndrome have been diagnosed, tests are done to find out if cancer cells have spread from the skin to other parts of the body.

The process used to find out if cancer has spread from the skin to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The following procedures may be used in the staging process:

• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the lymph nodes, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the lymph nodes, chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• Lymph node biopsy: The removal of all or part of a lymph node. A pathologist views the tissue under a microscope to look for cancer cells.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

• Through tissue. Cancer invades the surrounding normal tissue.
• Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
• Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

The following stages are used for mycosis fungoides and the Sézary syndrome:

Stage I

Stage I is divided into stage IA and stage IB as follows:

• Stage IA: Less than 10% of the skin surface is covered with patches and/or plaques.
• Stage IB: Ten percent or more of the skin surface is covered with patches and/or plaques.

Stage II

Stage II is divided into stage IIA and stage IIB as follows:

• Stage IIA: Any amount of the skin surface is covered with patches and/or plaques. Lymph nodes are enlarged but cancer has not spread to them.
• Stage IIB: One or more tumors are found on the skin. Lymph nodes may be enlarged but cancer has not spread to them.

Stage III

In stage III, nearly all of the skin is reddened and may have patches, plaques, or tumors. Lymph nodes may be enlarged but cancer has not spread to them.

Stage IV

Stage IV is divided into stage IVA and stage IVB as follows:

• Stage IVA: Most of the skin is reddened and any amount of the skin surface is covered with patches, plaques, or tumors. Cancer has spread to lymph nodes, and the lymph nodes may be enlarged.
• Stage IVB: Most of the skin is reddened and any amount of the skin surface is covered with patches, plaques, or tumors. Cancer has spread to other organs in the body. Lymph nodes may be enlarged and cancer may have spread to them.

Stages of mycosis fungoides and the Sézary syndrome may also have a B classification.

The B classification is based on how many abnormal lymphocytes are found in the blood.

Recurrent Mycosis Fungoides and the Sézary Syndrome

Recurrent mycosis fungoides and the Sézary syndrome are cancers that have recurred (come back) after they have been treated. The cancer may come back in the skin or in other parts of the body.

Treatment Option Overview

Key Points for This Section

There are different types of treatment for patients with mycosis fungoides and the Sézary syndrome cancer. Five types of standard treatment are used: Photodynamic therapy Radiation therapy Chemotherapy Other drug therapy Biologic therapy New types of treatment are being tested in clinical trials. High-dose chemotherapy and radiation therapy with stem cell transplant Patients may want to think about taking part in a clinical trial. Patients can enter clinical trials before, during, or after starting their cancer treatment. Follow-up tests may be needed.

There are different types of treatment for patients with mycosis fungoides and the Sézary syndrome cancer.

Different types of treatment are available for patients with mycosis fungoides and the Sézary syndrome. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Five types of standard treatment are used:

Photodynamic therapy

Photodynamic therapy is a cancer treatment that uses a drug and a certain type of laser light to kill cancer cells. A drug that is not active until it is exposed to light is injected into a vein. The drug collects more in cancer cells than in normal cells. For skin cancer, laser light is shined onto the skin and the drug becomes active and kills the cancer cells. Photodynamic therapy causes little damage to healthy tissue. Patients undergoing photodynamic therapy will need to limit the amount of time spent in sunlight.

In one type of photodynamic therapy, called psoralen and ultraviolet A (PUVA) therapy, the patient receives a drug called psoralen and then ultraviolet radiation is directed to the skin. In another type of photodynamic therapy, called extracorporeal photochemotherapy, the patient is given drugs and then some blood cells are taken from the body, put under a special ultraviolet A light, and put back into the body.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

Sometimes, total skin electron beam (TSEB) radiation therapy is used to treat mycosis fungoides and the Sézary syndrome. This is a type of radiation treatment in which the skin over the whole body is treated with rays of tiny particles called electrons.

The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Sometimes the chemotherapy is topical (applied to the skin in a cream or lotion.) The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Other drug therapy

Retinoids, are drugs related to vitamin A that can slow the growth of certain types of cancer cells. The retinoids may be taken by mouth or applied to the skin.

Biologic therapy

Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

Specific types of biologic therapy used in treating mycosis fungoides and the Sézary syndrome include the following:

• Monoclonal antibody therapy: A cancer treatment that uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.
• Interferon alfa: A substance that interferes with the division of cancer cells and can slow tumor growth.
• Interleukin-2: A substance that can improve the body's natural response to infection and disease.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site ⁵.

High-dose chemotherapy and radiation therapy with stem cell transplant

This treatment is a method of giving high doses of chemotherapy and radiation therapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the bone marrow or blood of the patient or a donor and are frozen and stored. After therapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options by Stage

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Treatment of stage I mycosis fungoides and the Sézary syndrome may include the following:

• PUVA therapy with or without interferon alfa.
• Radiation therapy to a single skin lesion or to all the skin on the body (TSEB).
• Radiation therapy to skin lesions, as palliative therapy to reduce tumor size or relieve symptoms and improve quality of life.
• Topical chemotherapy.
• Interferon alfa with or without topical chemotherapy.
• Retinoid therapy.
• Systemic chemotherapy. .

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage I mycosis fungoides/Sezary syndrome ⁶.

Treatment of stage II mycosis fungoides and the Sézary syndrome is palliative and may include the following:

• PUVA therapy with or without interferon alfa.
• Radiation therapy to all the skin on the body (TSEB).
• Radiation therapy to skin lesions, to reduce tumor size or relieve symptoms and improve quality of life.
• Topical chemotherapy.
• Interferon alfa with or without topical chemotherapy.
• Retinoid therapy.
• Systemic chemotherapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage II mycosis fungoides/Sezary syndrome ⁷.

Treatment of stage III mycosis fungoides and the Sézary syndrome is palliative and may include the following:

• PUVA therapy with or without systemic chemotherapy.
• PUVA therapy with or without interferon alfa.
• Extracorporeal photochemotherapy.
• Radiation therapy to all the skin on the body (TSEB).
• Radiation therapy to skin lesions, to reduce tumor size or relieve symptoms and improve quality of life.
• Systemic chemotherapy with one or more drugs, with or without topical chemotherapy.
• Topical chemotherapy.
• Biologic therapy (interferon alfa or interleukin-2) with or without topical chemotherapy.
• Retinoid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage III mycosis fungoides/Sezary syndrome ⁸.

Treatment of stage IV mycosis fungoides and the Sézary syndrome is palliative and may include the following:

• PUVA therapy with or without systemic chemotherapy.
• PUVA therapy with or without interferon alfa.
• Extracorporeal photochemotherapy with or without radiation therapy to all the skin on the body (TSEB).
• Radiation therapy to all the skin on the body, with or without systemic chemotherapy.
• Radiation therapy to skin lesions.
• Systemic chemotherapy with one or more drugs.
• Biologic therapy (interferon alfa or interleukin-2) with or without topical chemotherapy.
• Topical chemotherapy.
• Monoclonal antibody therapy.
• Retinoid therapy.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with stage IV mycosis fungoides/Sezary syndrome ⁹.

Treatment Options for Recurrent Mycosis Fungoides and the Sézary Syndrome

Treatment of recurrent mycosis fungoides and the Sézary syndrome may include the following:

• Radiation therapy to skin lesions or all the skin on the body (TSEB).
• PUVA therapy.
• Topical chemotherapy.
• A clinical trial of PUVA therapy with interferon alfa.
• A clinical trial of extracorporeal photochemotherapy.
• A clinical trial of radiolabeled monoclonal antibody therapy.
• A clinical trial of interleukin-2.
• A clinical trial of retinoid therapy.
• A clinical trial of chemotherapy.
• A clinical trial of high-dose chemotherapy with stem cell transplant.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent mycosis fungoides/Sezary syndrome ¹⁰.

To Learn More About Mycosis Fungoides and the Sézary Syndrome

For more information from the National Cancer Institute about mycosis fungoides and the Sézary syndrome, see the following:

• Biological Therapies for Cancer: Questions and Answers ¹¹
• Photodynamic Therapy for Cancer: Questions and Answers ¹²

For general cancer information and other resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview ¹³
• Understanding Cancer Series: Cancer ¹⁴
• Staging: Questions and Answers ¹⁵
• Chemotherapy and You: Support for People With Cancer ¹⁶
• Radiation Therapy and You: Support for People With Cancer ¹⁷
• Coping with Cancer ¹⁸
• Support and Resources ¹⁹
• Cancer Library ²⁰
• Information For Survivors/Caregivers/Advocates ²¹

Get More Information From NCI

Call 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

Chat online

The NCI's LiveHelp® ²² online chat service provides Internet users with the ability to chat online with an Information Specialist. The service is available from 9:00 a.m. to 11:00 p.m. Eastern time, Monday through Friday. Information Specialists can help Internet users find information on NCI Web sites and answer questions about cancer.

Write to us

For more information from the NCI, please write to this address:

NCI Public Inquiries Office

Suite 3036A

6116 Executive Boulevard, MSC8322

Bethesda, MD 20892-8322

Search the NCI Web site

The NCI Web site ²³ provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. For a quick search, use our “Best Bets” search box in the upper right hand corner of each Web page. The results that are most closely related to your search term will be listed as Best Bets at the top of the list of search results.

There are also many other places to get materials and information about cancer treatment and services. Hospitals in your area may have information about local and regional agencies that have information on finances, getting to and from treatment, receiving care at home, and dealing with problems related to cancer treatment.

Find Publications

The NCI has booklets and other materials for patients, health professionals, and the public. These publications discuss types of cancer, methods of cancer treatment, coping with cancer, and clinical trials. Some publications provide information on tests for cancer, cancer causes and prevention, cancer statistics, and NCI research activities. NCI materials on these and other topics may be ordered online or printed directly from the NCI Publications Locator ²⁴. These materials can also be ordered by telephone from the Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Changes to This Summary (05/06/2008)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

Several enhancements have been made to this summary to better explain certain medical concepts and to help readers find information about clinical trials. The following changes were made:

• Text describing the way cancer spreads in the body was added to the Stages ²⁵ section.
• Information about patients taking part in clinical trials and about follow-up tests was added to the Treatment Option Overview ²⁶ section.
• Links to ongoing clinical trials listed in NCI's PDQ Cancer Clinical Trials Registry were added to the Treatment Options ²⁷ section.
• A new section called "To Learn More ²⁸" was added. It includes links to more information about this type of cancer and about cancer in general.
• The "Get More Information from NCI" section (originally called "To Learn More") was revised.

About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site ²³. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site ²⁹. Descriptions of the trials are available in health professional and patient versions. Many cancer doctors who take part in clinical trials are also listed in PDQ. For more information, call the Cancer Information Service 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancerous, premalignant (likely to become cancer), or benign.

A type of protein made by plasma cells (a type of white blood cell) in response to an antigen (foreign substance). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Antibodies can work in several ways, depending on the nature of the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.

A substance that causes the immune system to make a specific immune response.

A white blood cell that comes from bone marrow. As part of the immune system, B cells make antibodies and help fight infections. Also called B lymphocyte.

A white blood cell that comes from bone marrow. As part of the immune system, B lymphocytes make antibodies and help fight infections. Also called B cell.

Treatment to boost or restore the ability of the immune system to fight cancer, infections, and other diseases. Also used to lessen certain side effects that may be caused by some cancer treatments. Agents used in biological therapy include monoclonal antibodies, growth factors, and vaccines. These agents may also have a direct antitumor effect. Also called immunotherapy, biotherapy, biological response modifier therapy, and BRM therapy.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.

Primary bone cancer is cancer that forms in cells of the bone. Some types of primary bone cancer are osteosarcoma, Ewing sarcoma, malignant fibrous histiocytoma, and chondrosarcoma. Secondary bone cancer is cancer that spreads to the bone from another part of the body (such as the prostate, breast, or lung).

The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.

Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord.

The smallest type of blood vessel. A capillary connects an arteriole (small artery) to a venule (small vein) to form a network of blood vessels in almost all parts of the body. The wall of a capillary is thin and leaky, and capillaries are involved in the exchange of fluids and gases between tissues and the blood.

A flexible tube used to deliver fluids into or withdraw fluids from the body.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

An x-ray of the structures inside the chest. An x-ray is a type of high-energy radiation that can go through the body and onto film, making pictures of areas inside the chest, which can be used to diagnose disease.

A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called a clinical study.

CBC. A test to check the number of red blood cells, white blood cells, and platelets in a sample of blood. Also called blood cell count and CBC.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

Computed tomography scan. A series of detailed pictures of areas inside the body taken from different angles; the pictures are created by a computer linked to an x-ray machine. Also called computed tomography scan, computerized tomography, computerized axial tomography scan, and CAT scan.

To heal or restore health; a treatment to restore health.

Having to do with the skin.

The process of identifying a disease, such as cancer, from its signs and symptoms.

Deoxyribonucleic acid. The molecules inside cells that carry genetic information and pass it from one generation to the next. Also called deoxyribonucleic acid.

The amount of medicine taken, or radiation given, at one time.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.

A procedure in which blood is removed from the body and treated with ultraviolet light and drugs that become active when exposed to light. The blood is then returned to the body. It is being studied in the treatment of some blood and bone marrow diseases and graft-vs-host disease (GVHD). Also called photopheresis.

Monitoring a person's health over time after treatment. This includes keeping track of the health of people who participate in a clinical study or clinical trial for a period of time, both during the study and after the study ends.

The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.

The substance inside red blood cells that binds to oxygen in the lungs and carries it to the tissues.

An intensive drug treatment to kill cancer cells, but that also destroys the bone marrow and can cause other severe side effects. High-dose chemotherapy is usually followed by bone marrow or stem cell transplantation to rebuild the bone marrow.

The complex group of organs and cells that defends the body against infections and other diseases.

A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose specific types of leukemia and lymphoma by comparing the cancer cells to normal cells of the immune system.

Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.

A method of putting fluids, including drugs, into the bloodstream. Also called intravenous infusion.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A biological response modifier (a substance that can improve the body's natural response to infections and other diseases). Interferons interfere with the division of cancer cells and can slow tumor growth. There are several types of interferons, including interferon-alpha, -beta, and -gamma. The body normally produces these substances. They are also made in the laboratory to treat cancer and other diseases.

A type of radiation therapy in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near a tumor. Also called brachytherapy, radiation brachytherapy, and implant radiation therapy.

Cancer that has spread beyond the layer of tissue in which it developed and is growing into surrounding, healthy tissues. Also called infiltrating cancer.

A device that concentrates light into an intense, narrow beam used to cut or destroy tissue. It is used in microsurgery, photodynamic therapy, and for a variety of diagnostic purposes.

An area of abnormal tissue. A lesion may be benign (noncancerous) or malignant (cancerous).

Cancer that starts in blood-forming tissue such as the bone marrow and causes large numbers of blood cells to be produced and enter the bloodstream.

The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.

A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

A lymphocyte that has gotten larger after being stimulated by an antigen. Lymphoblast also refers to an immature cell that can develop into a mature lymphocyte.

A type of white blood cell. Lymphocytes have a number of roles in the immune system, including the production of antibodies and other substances that fight infections and other diseases.

Referring to lymphocytes, a type of white blood cell. Also refers to tissue in which lymphocytes develop.

Cancer that begins in cells of the immune system. There are two basic categories of lymphomas. One kind is Hodgkin lymphoma, which is marked by the presence of a type of cell called the Reed-Sternberg cell. The other category is non-Hodgkin lymphomas, which includes a large, diverse group of cancers of immune system cells. Non-Hodgkin lymphomas can be further divided into cancers that have an indolent (slow-growing) course and those that have an aggressive (fast-growing) course. These subtypes behave and respond to treatment differently. Both Hodgkin and non-Hodgkin lymphomas can occur in children and adults, and prognosis and treatment depend on the stage and the type of cancer.

A diagnostic indication that disease may develop.

The spread of cancer from one part of the body to another. A tumor formed by cells that have spread is called a “metastatic tumor” or a “metastasis.” The metastatic tumor contains cells that are like those in the original (primary) tumor. The plural form of metastasis is metastases (meh-TAS-tuh-SEEZ).

A type of protein made in the laboratory that can locate and bind to substances in the body, including tumor cells. There are many kinds of monoclonal antibodies. Each monoclonal antibody is made to find one substance. Monoclonal antibodies are being used to treat some types of cancer and are being studied in the treatment of other types. They can be used alone or to carry drugs, toxins, or radioactive materials directly to a tumor.

A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, nuclear magnetic resonance imaging, and NMRI.

A type of non-Hodgkin lymphoma that first appears on the skin and can spread to the lymph nodes or other organs such as the spleen, liver, or lungs.

In mycosis fungoides, an area of skin that is thickened, raised, red, scaly, and itchy.

Having to do with or resembling the bone marrow. May also refer to certain types of hematopoietic (blood-forming) cells found in the bone marrow. Sometimes used as a synonym for myelogenous; for example, acute myeloid leukemia and acute myelogenous leukemia are the same disease.

The National Cancer Institute, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. The National Cancer Institute conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the National Cancer Institute Web site at http://www.cancer.gov. Also called NCI.

NK cell. A type of white blood cell that contains granules with enzymes that can kill tumor cells or microbial cells. Also called large granular lymphocyte and NK cell.

NCI, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. It conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the NCI Web site at http://www.cancer.gov. Also called National Cancer Institute.

Any of a large group of cancers of the immune system. Non-Hodgkin lymphomas can occur at any age and are often marked by enlarged lymph nodes, fever, and weight loss. There are many different types of non-Hodgkin lymphoma, which can be divided into aggressive (fast-growing) and indolent (slow-growing) types and can be classified as either B-cell or T-cell non-Hodgkin lymphoma. B-cell non-Hodgkin lymphomas include Burkitt lymphoma, diffuse large B-cell lymphoma, follicular lymphoma, immunoblastic large cell lymphoma, precursor B-lymphoblastic lymphoma, and mantle cell lymphoma. T-cell non-Hodgkin lymphomas include mycosis fungoides, anaplastic large cell lymphoma, and precursor T-lymphoblastic lymphoma. Lymphomas related to lymphoproliferative disorders following bone marrow or stem cell transplantation are usually B-cell non-Hodgkin lymphomas. Prognosis and treatment depend on the stage and type of disease. Also called NHL.

A part of the body that performs a specific function. For example, the heart is an organ.

Treatment given to relieve the symptoms and reduce the suffering caused by cancer and other life-threatening diseases. Palliative cancer therapies are given together with other cancer treatments, from the time of diagnosis, through treatment, survivorship, recurrent or advanced disease, and at the end of life.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

The lower part of the abdomen, located between the hip bones.

Blood circulating throughout the body.

A procedure in which a sample of blood is viewed under a microscope to count different circulating blood cells (red blood cells, white blood cells, platelets, etc.) and see whether the cells look normal.

Treatment with drugs that become active when exposed to light. These activated drugs may kill cancer cells.

An exam of the body to check for general signs of disease.

A type of blood cell that helps prevent bleeding by causing blood clots to form. Also called a thrombocyte.

A phase of mycosis fungoides in which a patient has areas of red, scaly, itchy skin on areas of the body that are usually not exposed to sun. This is early-phase mycosis fungoides, but it is hard to diagnose the rash as mycosis fungoides during this phase. The premycotic phase may last from months to decades.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

Cancer that is growing, spreading, or getting worse.

A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.

A substance from plants that is sensitive to light (or can be activated by light). Psoralens are used together with UV light to treat psoriasis, vitiligo, and skin nodules of cutaneous T-cell lymphoma. They are also being studied in the treatment of graft-versus-host disease. Psoralen is a type of furocoumarin. An example of a psoralen is methoxsalen.

A type of photodynamic therapy used to treat skin conditions such as psoriasis, vitiligo, and skin nodules of cutaneous T-cell lymphoma. The patient receives psoralen (a drug that becomes active when it is exposed to light) by mouth or applied to the skin, followed by ultraviolet A radiation. Psoralen and ultraviolet A therapy may increase the risk of getting skin cancer. Also called PUVA therapy.

A type of photodynamic therapy used to treat skin conditions such as psoriasis, vitiligo, and skin nodules of cutaneous T-cell lymphoma. The patient receives psoralen (a drug that becomes active when it is exposed to light) by mouth or applied to the skin, followed by ultraviolet A radiation. PUVA therapy may increase the risk of getting skin cancer. Also called psoralen and ultraviolet A therapy.

The overall enjoyment of life. Many clinical trials assess the effects of cancer and its treatment on the quality of life. These studies measure aspects of an individual’s sense of well-being and ability to carry out various activities.

Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called radiotherapy and irradiation.

Giving off radiation.

A small, radioactive pellet that is placed in or near a tumor. Cancer cells are killed by the energy given off as the radioactive material breaks down and becomes more stable.

Any compound that has been joined with a radioactive substance.

A molecule inside or on the surface of a cell that binds to a specific substance and causes a specific physiologic effect in the cell.

To come back or to return.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrent cancer.

Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.

RBC. A cell that carries oxygen to all parts of the body. Also called RBC and erythrocyte.

Treatment with anticancer drugs directed to a specific area of the body.

Vitamin A or a vitamin A-like compound.

A form of cutaneous T-cell lymphoma, a cancerous disease that affects the skin.

A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spinal column encloses the spinal cord and the fluid surrounding the spinal cord. Also called spine, backbone, and vertebral column.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

Stage I is divided into stages IA and IB. In stage IA, less than 10% of the skin surface is covered with patches and/or plaques (areas of abnormal tissue). In stage IB, 10% or more of the skin surface is covered with patches and/or plaques. Stage I may also be classified based on how many abnormal lymphocytes (white blood cells) are found in the blood.

Stage II is divided into stages IIA and IIB. In stage IIA, any amount of the skin surface is covered with patches and/or plaques (areas of abnormal tissue). Lymph nodes are enlarged but cancer has not spread to them. In stage IIB, one or more tumors (lumps of malignant cells) are found on the skin. Lymph nodes may be enlarged but cancer has not spread to them. Stage II may also be classified based on how many abnormal lymphocytes (white blood cells) are found in the blood.

In stage III, nearly all of the skin is reddened and there may be patches, plaques, or tumors. Lymph nodes may be enlarged but cancer has not spread to them. Stage III may also be classified based on how many abnormal lymphocytes (white blood cells) are found in the blood.

Stage IV is divided into stages IVA and IVB. In stage IVA, most of the skin is reddened, and any amount of the skin is covered with patches and/or plaques (areas of abnormal tissue) or tumors (lumps of malignant cells). Cancer has spread to lymph nodes, and the lymph nodes may be enlarged. In stage IVB, most of the skin is reddened, and any amount of the skin is covered with lesions or tumors. Cancer has spread to other organs in the body. Lymph nodes may be enlarged and cancer may have spread to them. Stage IV may also be classified based on how many abnormal lymphocytes (white blood cells) are found in the blood.

Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called standard of care or best practice.

A cell from which other types of cells develop. For example, blood cells develop from blood-forming stem cells.

A method of replacing immature blood-forming cells that were destroyed by cancer treatment. The stem cells are given to the person after treatment to help the bone marrow recover and continue producing healthy blood cells.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.

Treatment with anticancer drugs that travel through the blood to cells all over the body.

One type of white blood cell that attacks virus-infected cells, foreign cells, and cancer cells. T cells also produce a number of substances that regulate the immune response. Also called T lymphocyte.

Treatment.

A group or layer of cells that work together to perform a specific function.

On the surface of the body.

Treatment with anticancer drugs in a lotion or cream applied to the skin.

TSEB radiation therapy. A type of radiation therapy using electrons that is directed at the entire surface of the body. This type of radiation goes into the outer layers of the skin, but does not go deeper into tissues and organs below the skin. Also called TSEB radiation therapy.

A poison produced by certain animals, plants, or bacteria.

Total skin electron beam radiation therapy. A type of radiation therapy using electrons that is directed at the entire surface of the body. This type of radiation goes into the outer layers of the skin, but does not go deeper into tissues and organs below the skin. Also called total skin electron beam radiation therapy.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancerous), or malignant (cancerous). Also called neoplasm.

The formation of a break on the skin or on the surface of an organ. An ulcer forms when the surface cells die and are cast off. Ulcers may be associated with cancer and other diseases.

Invisible rays that are part of the energy that comes from the sun. UV radiation also comes from sun lamps and tanning beds. UV radiation can damage the skin and cause melanoma and other types of skin cancer. UV radiation that reaches the Earth's surface is made up of two types of rays, called UVA and UVB rays. UVB rays are more likely than UVA rays to cause sunburn, but UVA rays pass deeper into the skin. Scientists have long thought that UVB radiation can cause melanoma and other types of skin cancer. They now think that UVA radiation also may add to skin damage that can lead to skin cancer and cause premature aging. For this reason, skin specialists recommend that people use sunscreens that reflect, absorb, or scatter both kinds of UV radiation. Also called UV radiation.

A blood vessel that carries blood to the heart from tissues and organs in the body.

In medicine, a very simple microorganism that infects cells and may cause disease. Because viruses can multiply only inside infected cells, they are not considered to be alive.

One of several vitamins that the body needs in small amounts to function and stay healthy. Vitamin A is needed for vision, bone growth, reproduction, growth of epithelium (cells that line the internal and external surfaces of the body), and fighting infections. It is fat-soluble (can be stored in the body’s fatty tissue). Foods with vitamin A include fish oils and liver, egg yolks, and whole milk dairy products from animals. It also can be made in the body from a substance found in fruits and vegetables such as cantaloupes, carrots, spinach, and sweet potatoes. Vitamin A is being studied in the prevention and treatment of some types of cancer. Also called retinol.

Refers to a blood cell that does not contain hemoglobin. White blood cells include lymphocytes, neutrophils, eosinophils, macrophages, and mast cells. These cells are made by bone marrow and help the body fight infections and other diseases. Also called WBC.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.