Digestive System Melanoma

Other Names: Melanoma of the gastrointestinal tract; Melanoma of the GI tract; Malignant melanoma of the gastrointestinal tract; primary gastrointestinal melanoma

Digestive system melanoma refers to a melanoma starting in the stomach, intestines, salivary glands, mouth, esophagus, liver, pancreas, gallbladder, or rectum. Melanoma is a disease in which malignant (cancer) cells form in the melanocytes. Melanocytes are commonly found in the skin and are the cells that give the skin color. While it is not uncommon for melanomas to start in the skin and later spread to other parts of the body (metastasize), melanomas originating in the gastrointestinal tract are rare. The most frequently reported site is in the esophagus and anorectum.

Cause

The cause of digestive system melanoma is not well understood.Some researchers theorize that it may have originated from an undetected primary tumor. Theories include: The cancer originated from a undetectable primary tumor that spontaneously (naturally) regressed on its own The cancer originated from a primary tumor that is so small it can not be detected using standard clinical and laboratory investigations Early [[melanocyte cells] (not usually found in the digestive system) lost their way during the development of the baby in the womb, and that these misplaced cells later became cancerous

Symptoms

Symptoms of digestive system melanoma can vary from person to person. Symptoms tend to be non-specific including: nausea, vomiting, stomachache, fatigue, hemorrhage (broken blood vessels), blood in stool, and anemia (low red blood cell count).

Diagnosis

A variety of imaging tests may be involved in the initial detection of the tumor, including: video capsule endoscopy, ultrasound, PET scan, and CT scan. The tumor is confirmed by surgical resection. Careful study of tissue samples from the tumor under a microscope will show the same immunohistochemical characteristics of skin melanomas. Once this has been established, the following are proposed diagnostic criteria for primary melanoma of the small intestine: I. The identification of a single solitary melanoma in the mucosa of the digestive system

II. The presence of other melanoma in the surrounding areas of the digestive system

III. The absence of cutaneous melanoma or other atypical skin tumors in the melanocytes

Treatment

Treatment of digestive system melanoma is dependent on the spread of the cancer and where it is located. Treatment might include surgery to remove the tumor and portion of the gastrointestinal tract involved, chemotherapy, and immunotherapy. The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Aldesleukin (Brand name: Proleukin)

Prognosis

The prognosis for individuals with digestive system melanoma varies from person to person. Important prognostic factors include if metastases are present at the time of diagnosis. Studies suggest that the average survival after complete removal of the tumor is about 48.9 months. The longest reported survival after diagnosis is 21 years.

NIH genetic and rare disease info

• NIH info on Digestive System Melanoma

Digestive System Melanoma is a rare disease.

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