$Unique_ID{BRK03988} $Pretitle{} $Title{Melanoma, Malignant} $Subject{Melanoma, Malignant Melanoma Nevus Pigmentosa Melanocarcinoma Melanoblastoma Melanotic Carcinoma Melanosarcoma Melanoepithelioma Melanoscirrhus Acral Lentiginous Melanoma Juvenile Melanoma Malignant Lentigo (Melanoma) Basal Cell Carcinoma Squamous Cell Carcinoma Kaposi's Sarcoma } $Volume{} $Log{} Copyright (C) 1989, 1990, 1992 National Organization for Rare Disorders, Inc. 684: Melanoma, Malignant ** IMPORTANT ** It is possible that the main title of the article (Malignant Melanoma) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Melanoma Nevus Pigmentosa Melanocarcinoma Melanoblastoma Melanotic Carcinoma Melanosarcoma Melanoepithelioma Melanoscirrhus Disorder Subdivisions: Acral Lentiginous Melanoma Juvenile Melanoma Malignant Lentigo (Melanoma) Information on the following diseases can be found in the Related Disorders section of this report: Basal Cell Carcinoma Squamous Cell Carcinoma Kaposi's Sarcoma General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Malignant Melanoma is a common skin cancer that arises from the melanin cells of the upper layer of the skin (epidermis) or from similar cells that can be found in moles (nevi). This type of skin cancer may send down roots into deeper layers of the skin. Some of these microscopic roots can spread (metastasize) causing new tumor growths in vital organs of the body. Symptoms In early stages most melanomas do not produce any specific symptoms. Later they may appear as a lesion that does not heal, or an existing mole that shows changes in size or color. A physician should be consulted when any lesion, pigmented or not, becomes itchy, burns, softens or hardens, forms a scab, bleeds, becomes surrounded by a reddened or inflamed area, changes color, size or shape. Disorder Subdivisions: Acral Lentiginious melanoma is a malignant skin cancer that occurs in areas that are not excessively exposed to sunlight and where hair follicles are absent. Juvenile Melanoma is a benign, elevated, pink to purplish-red papule, with a slightly scaly surface. It usually appears on the face, especially the cheeks. This type of melanoma most often occurs before puberty and has been mistaken for malignant melanoma. Malignant Lentigo (Melanoma) is a precancerous area on the skin, that resembles a freckle. It can be brown or black in color, irregular in shape, and it usually occurs on the face. This type of Melanoma occurs most often in older people. Causes The exact cause of Malignant Melanoma is unknown. Excessive exposure to the sun, particularly before puberty, and living in areas that are closer to the sun, increases the risk of developing skin cancer. There may be a genetic predisposition for malignant melanoma which may be transmitted through autosomal dominant genes. Human traits including the classic genetic diseases are the product of the interaction of two genes for that condition, one received from the father and one from the mother. In dominant disorders a single copy of the disease gene (received from either the mother or father) will be expressed "dominating" the other normal gene and resulting in appearance of the disease. The risk of transmitting the disorder from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child. A genetic predisposition to an illness means that some people may carry the defective gene but never get the disorder unless something in the environment triggers the disease process. Affected Population Malignant Melanoma affects males and females in equal numbers. The incidence of these types of skin cancers is increasing at a far faster rate than any other cancers. The risk of melanoma is higher in Caucasians than in those of more darkly pigmented races. It is even a greater risk for those with blue eyes and fair complexion. Related Disorders Symptoms of the following disorders can be similar to those of Malignant Melanoma. Comparisons may be useful for a differential diagnosis: Basal Cell Carcinoma is a common skin cancer. It may appear as small, shiny, firm nodules; ulcerated, crusted lesions; or flat, scar-like hardened patches which may bleed. This type of skin cancer is difficult to differentiate from psoriasis or localized dermatitis without a biopsy. Squamous Cell Carcinomas usually appear on sun-exposed areas of the skin, but may occur anywhere on the body. The lesions begin as a small red elevation or patch with a scaly or crusted surface. They may become nodular, sometimes with a warty surface. In some, the bulk of the lesion may lie below the level of the surrounding tissue. A biopsy is essential to diagnose this disorder. Kaposi's Sarcoma may appear as small pigmented (tan to purple) papules, plaques, nodules, tumors or ulcers. This type of skin cancer can infiltrate the body, involving the oropharynx and gastrointestinal tract, disseminating to other organs such as the liver, lung and bone. Chemotherapy has been helpful in treating Kaposi's Sarcoma. Until the last 10 years it was seen mostly in older men of Ashkenazi Jewish or Mediterranean descent, and those with a compromised immune system. The more recent increased incidence of Kaposi's Sarcoma is due to AIDS (Acquired Immunodeficiency Syndrome); about 30% of those with AIDS will also get Kaposi's Sarcoma. Therapies: Standard The treatment for Malignant Melanoma depends on the level, stage and location of the skin cancer at the time of diagnosis. For stage 1 disease, surgery to remove the affected area is a wide excision with 5-cm margins around the lesion. In some locations, such as the face, smaller margins must be accepted. If the cancer has progressed to the lymph nodes, Stage 2, a complete removal of the involved nodes (lymphadenectomy) must be done. Regular follow-ups are advisable and should include an annual chest X-Ray. For patients with metastatic disease, certain chemotherapeutic agents (drugs), are being used alone or in combination with other drugs. Decarbazine, used in this manner has resulted in a temporary remission for some patients. A course of treatment that includes high-dose alkylating agents such as cyclophosphamide, cisplatin, and carmustine, may also be effective as a treatment for Malignant Melanoma. Therapies: Investigational at the present time there are several new drug studies dealing with Malignant Melanoma. Scientists are trying to develop drugs to enhance the immune system, including a vaccine. The drug Interferon, used alone or in combination with other chemotherapeutic agents, is also being tested. Autologous bone marrow transplants are being done experimentally for treatment of Malignant Melanoma. More research must be conducted to determine long-term safety and effectiveness of these drugs and procedures. The Office of Orphan Products Development has awarded a New Grant Award for the year 1990 to Dr. Jean Claude Bystryn of New York Medical Center, New York, NY, for clinical trial work of a Polyvalent Antigen Vaccine for treatment of Melanoma. Clinical trials are underway to study Interleukin-2 and Tumor- Infiltrating Lymphocytes in patients with Melanoma. Interested persons may wish to contact: Timothy J. Eberlein, M.D. Brigham and Women's Hospital 75 Francis St. Boston, MA 02115 (617) 732-6799 to see if further patients are needed for this research. The orphan product Melphalan, trade name Alkeran for injection, is being tested by the FDA as a treatment for Metastic Melanoma. The product is being sponsored by Burroughs Wellcome, Co., 3030 Cornwallis Rd., Research Triangle Park, NC, 27709. This disease entry is based upon medical information available through April 1992. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Malignant Melanoma, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Melanoma Foundation 750 Menlo Avenue Suite 250 Menlo Park, CA 94025 (415) 326-3974 Helping Hand 12 Arlington St. Portland, ME 04101 The Skin Cancer Foundation 475 Park Avenue South New York, NY 10016 (212) 725-5176 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Cancer Institute 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give the public, cancer patients and families, and health professionals quick and easy access to many types of information vital to patients with this and many other types of cancer. To gain access to this service, call: Cancer Information Service (CIS) 1-800-4-CANCER In Washington, DC and suburbs in Maryland and Virginia, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect) For genetic information and genetic counseling referrals, please contact: March of Dimes Birth Defects Foundation 1275 Mamaroneck Avenue White Plains, NY 10605 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 References MENDELIAN INHERITANCE IN MAN, 8th ed.: Victor A. McKusick; Johns Hopkins University Press, 1986. Pp. 485. INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 1109, 1111, 1372. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in- chief; Merck Sharp & Dohme Research Laboratories., 1982. Pp. 1164. IMMUNOTHERAPY FOR MALIGNANT MELANOMA, VACCINES. JC Bystryn; MEL LET (Vol. 4; No. 2 1986). CHANGING TRENDS IN MELANOMA. CM Balch, M.D.; ed.-in-chief; MEL LET (Vol. 5, No. 1, 1987). MALIGNANT MELANOMA. TREATMENT WITH HIGH DOSE COMBINATION ALKYLATING AGENT CHEMOTHERAPY AND AUTOLOGOUS BONE MARROW SUPPORT. TC Shea; ARCH DERMATOL, (June 1988; 124(6)). Pp. 878-884.