MUCOSAL MALIGNANT MELANOMA

ALTERNATE NAMES

Primary Mucosal Melanomas; Extracutaneous Malignant Melanomas (EMM); Primary Sinonasal Mucosal Melanoma (SNMM); Anorectal Melanoma (ARM); Mucosal Melanoma of the Head and Neck; Melanoma of the Esophagus; Melanoma of the Male Genito-Urinary Tract; Vulval Melanoma; Vaginal Melanoma; Mucosal Melanoma; Buccal Melanoma; Oral Melanoma

DESCRIPTION

Mucosal Malignant Melanomas are rare, biologically aggressive neoplasms that are distinguished from cutaneous (skin) melanomas in that they originate in the mucus membranes lining the respiratory, digestive and genitourinary tracts or in the eyes as well as in the cerebral meninges. Because they are not easily detected, they are often diagnosed very late in their course and have a poor prognosis for recovery using standard therapies. The disorders occur primarily in elderly people and affect both genders equally. The most common types of mucosal malignant melanomas occur in the head and neck.

DIAGNOSTIC

TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Definitive diagnostic lab testing: A biopsy of the pigmented lesion is performed and reviewed by a pathologist. Additionally, lymph node biopsy of the regional nodes may be performed to determine staging.

Other supporting lab testing, including X-rays: To stage the extent of melanoma, CT scan, MRI, PET scan or other imaging study may be performed.

Physical findings: The nasal cavity is the most common location of mucosal melanoma within the head and neck area; and it may present with epistaxis, nasal obstruction, or facial pain. Those lesions involving the oral cavity usually present as a painless mass with ulceration/bleeding. Involvement of the genital/urinary tracts may present with abnormal discharge or bleeding/hematuria. The rectal area may involve pain or a mass.

ICD-9: Coded according to site of malignant neoplasm

ONSET AND PROGRESSION

This is a rare condition with a peak incidence in patients aged 60-80 years. Unlike melanomas involving the skin, exposure to sunlight is not a risk factor. Prognosis is poor and survival times vary with the location of the melanoma and stage of the disease at the time of diagnosis.

TREATMENT

Treatment involves surgical resection and may include adjuvant radiation and chemotherapy. As there is a high incidence of systemic disease in these cases, a CT/PET scan is indicated prior to radical surgery. Metastatic disease is not curable.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for Evaluation: Pathology report and documentation of mucosal melanoma metastasis

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

13.29C

Medical Equals