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$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
     [phone]

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
     [phone]

     Melanoma Foundation
     750 Menlo Avenue
     Suite 250
     Menlo Park, CA 94025
     [phone]

     Helping Hand
     12 Arlington St.
     Portland, ME  04101

     The Skin Cancer Foundation
     475 Park Avenue South
     New York, NY 10016
     [phone]

     American Cancer Society
     1599 Clifton Rd., NE
     Atlanta, GA  30329
     [phone]

     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, [phone] (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
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

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.