Rare variants of malignant melanoma

The personal experience with 5 rare types of malignant melanoma is reviewed to point out some of the practical problems in the diagnosis and management of these tumors. The rare forms discussed are conjunctival, nasal, oral, vulvar, and penile melanomas. All pigmented lesions in the oral cavity, but not the penis or vulva, should be prophylactically excised as lesions in the mouth have a higher malignant potential. Local excision of all 5 forms of primary melanomas, no matter how locally advanced they may be, is the sole treatment. Nevertheless, anatomic constraints often preclude surgery with generous margins and consequently local recurrence, particularly for conjunctival, nasal, and oral primary lesions, is usually the major first failure in treatment. Lymph node dissection is only performed if the regional nodes are palpable at the time of first presentation. Elective lymph node dissections are not performed since the patients are often elderly, the lymphatic drainage is usually ambiguous or multiple, and the disease tends to spread hematogenously rather than lymphatically. Surgery still remains the cornerstone of treatment for these rare forms of melanoma but prognosis is very poor since surgery is often a palliative rather than a curative measure. Improved survival may depend on identifying more effective chemotherapeutic and immunologic agents.