$Unique_ID{BRK03557} $Pretitle{} $Title{Cancers, Skin, General} $Subject{Cancers Skin General Melanoma Non-Melanoma Melanoma Malignant Basal Cell Carcinoma Squamous Cell Carcinomas Acral Lentiginious Melanoma Juvenile Melanoma Kaposi's Sarcoma Malignant Lentico Melanoma Dysplastic Nevus Syndrome Xeroderma Pigmentosum} $Volume{} $Log{} Copyright (C) 1990, 1991, 1992 National Organization for Rare Disorders, Inc. 786: Cancers, Skin, General ** IMPORTANT ** It is possible that the main title of the article (Skin Cancers, General) 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 Non-Melanoma Disorder Subdivisions: Melanoma, Malignant Basal Cell Carcinoma Squamous Cell Carcinomas Acral Lentiginious Melanoma Juvenile Melanoma Kaposi's Sarcoma Malignant Lentico Melanoma Information on the following diseases can be found in the Related Disorders section of this report: Dysplastic Nevus Syndrome Xeroderma Pigmentosum 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. There are many different types of Skin Cancer. Combined together all types of Skin Cancer represent the most prevalent type of cancer. Most skin cancers are characterized by changes in the color or texture of the skin, but some types begin under the skin where they can spread to other parts of the body. Malignant melanoma is the most dangerous of this type of skin cancer. Symptoms Skin Cancer is the most common of all cancers. The incidence of Skin Cancer is rising faster than any other type of cancer, increasing more than 30% percent in recent years. The most dangerous form of skin cancer is malignant melanoma. It occurs below the surface of the skin and spreads easily to other locations in the body. Malignant Melanoma may appear as a lesion that does not heal, or an existing mole that shows changes in size and color. Non- melanoma type skin cancers are mainly basal cell and squamous cell carcinomas that occur on the skins surface and are easier to locate and treat. They may appear as small, shiny nodules or ulcerated crusted lesions. They may also appear as flat, scar-like hardened patches which bleed, or patchy and scaly elevations on the skin. If left untreated both types of skin cancer can spread (metastasize) to other parts of the body causing further disease. Causes Most types of skin cancer are caused by over-exposure to the sun's harmful ultra violet rays. B type ultraviolet rays are related to onset of malignant melanoma. However, the direct relationship is not fully understood. Life style and skin coloring also contribute to the development of skin cancer. Some scientists believe that there may be a genetic predisposition for some forms of skin cancer but more study is necessary to determine exactly why this happens. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease. Affected Population Skin Cancers affect males and females in equal numbers. However, persons with fair skin and hair, and persons living closer to the equator and/or at higher elevations, have a higher risk of developing skin cancer than others. The various types of skin cancers, when combined, represent the most prevalent type of cancer. The following disorders are the most common types of Skin Cancers. 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. A physician should be consulted when any lesions, 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. (For more information on this disorder, choose "Malignant Melanoma" as your search term in the Rare Disease Database). 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 Carcinoma usually appears 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 cancer may lie below the level of the surrounding tissue. A biopsy is essential to diagnose this disorder. Acral Lentiginious Melanoma is a malignant skin cancer that usually occurs in areas or the body 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 persons. Kaposi's Sarcoma is a type of skin cancer usually found in people whose immune system is dysfunctional. It appears as small pigmented (tan to purple) papules, plaques, nodules, tumors or ulcers on the skin. This type of skin cancer can infiltrate the body, involving the throat and gastrointestinal tract, disseminating to other organs such as the liver, lungs 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 in those with a compromised immune system. The more recent increased incidence of Kaposi's Sarcoma is due to AIDS; about 30% of those with AIDS will get Kaposi's Sarcoma. (For more information on this disorder, choose "Kaposi's Sarcoma" or "AIDS" as your search term in the Rare Disease Database). Related Disorders Symptoms of the following disorders can be similar to those of Skin Cancer. Comparisons may be useful for a differential diagnosis: Dysplastic Nevus Syndrome is a rare disorder that usually starts during adulthood. The disorder is characterized by large moles which are reddish- brown to pink in color. The moles have an irregular border. The presence of dust-like melanin, which gives the moles their color, and abnormally large nuclei of skin cells called melanocytes (all visible under the microscope), are characteristic of Dysplastic Nevus Syndrome. The mole-like tumors may spread to adjacent parts of the skin, or through the blood and lymph circulation to other organs. Certain changes in the melanocyte nuclei indicate when Dysplastic Nevus Syndrome may be changing to Malignant Melanoma. (For more information on this disorder, choose "Dysplastic Nevus Syndrome" as your search term in the Rare Disease Database). Xeroderma Pigmentosum is a rare autosomal recessive hereditary skin disorder which begins during early childhood. It is characterized by a defect in the ability of certain connective tissue cells to repair skin damaged by the ultraviolet rays of the sun. The skin of people with Xeroderma Pigmentosum is markedly hypersensitive to sunlight. Plaques and blisters develop when exposed to the sun. Many persons with XP go on to develop skin cancer. (For more information on this disorder, choose "Xeroderma" as your search term in the Rare Disease Database). Therapies: Standard Treatment of Skin Cancer depends on the depth, level, type, stage and location of the lesion at the time of diagnosis. Surgery to remove the affected area is usually the first step in treatment. If the cancer has progressed beyond the skin and is affecting other organs then the physician must make the determination as to further treatment. Chemotherapy (drugs) are used in some courses of treatment, as is radiation. Preventative measures are the most helpful. Many dermatologists recommend that parents begin the protection of their children against the suns harmful rays as soon as they are old enough to be playing out of doors. A sun blocking lotion is the most useful protection in keeping the harmful ultra violet rays of the sun off of a person's skin. Therapies: Investigational Scientists are studying many new drugs to treat various types of skin cancers. Melaccine, antimelanoma antibody and Technetium Tc 99m are being studied for the treatment of melanoma. For the treatment of Kaposi's Sarcoma interferon alfa, nl, interferon alfa 2a and interferon alfa 2b are being studied. Autologous bone marrow transplants are being done experimentally for treatment of Malignant Melanoma but this procedure carries a very high risk. Scientists are searching for ways to enhance the immune system which may be helpful for many types of cancer. More research is necessary to determine the long-term safety and effectiveness of these and other new drugs and procedures being tested for skin cancers. Another orphan biologic being tested for use in treating Malignant Melanoma patients is Interferon Beta (Recombinant) (r-IFN)-beta). The manufacturer is Biogen, Inc. 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 as a treatment for Metastic Melanoma. The product is being sponsored by Burrough 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 on General Skin Cancer, please contact: National Organization for Rare Disorders P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The Skin Cancer Foundation 475 Park Avenue, South New York, NY 10016 212-725-5176 Melanoma Foundation 750 Menlo Avenue, Suite 250 Menlo Park, CA 94025 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 MD and VA, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect). References CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp. 1094, 2336-2340. MELANOMA METASTATIC TO THE GASTROINTESTINAL TRACT. J. Kruse, et al.; Am Fam Physician, (January, 1990, issue 41 (1)). Pp. 165-168. PROGNOSIS OF THICK CUTANEOUS MELANOMA OF THE TRUNK AND EXTREMITY. D. Coit, et al.; Arch Surg, (March, 1990, issue 125 (3)). Pp. 322-326. CLINICAL CHARACTERISTICS OF MALIGNANT MELANOMAS DEVELOPING IN PERSONS WITH DYSPLASTIC NEVI. J.K. River, Cancer, (March 1, 1990, issue 65 (5)). Pp. 1232-1236. MALIGNANT MELANOMA OF SOFT PARTS (CLEAR CELL SARCOMA). A STUDY OF 17 CASES, WITH EMPHASIS ON PROGNOSTIC FACTORS. A.S. Sara, et al,; Cancer (January 15, 1990, issue 65 (2)). Pp. 367-374.