Primary mucosal desmoplastic melanoma of the head and neck.

Overview

BACKGROUND: Desmoplastic variants of melanoma rarely affect the mucosa. METHOD: The clinicopathologic features and p53 status of seven primary mucosal desmoplastic melanomas of the oral and nasal cavity were studied. RESULTS: The patients included six men and one woman (median age, 61 years; range, 23-74 years). Desmoplastic melanoma involved the lip in two, alveolus in three, buccal mucosa in one, and nasal vestibule in one patient. Four tumors infiltrated up to lamina propria, and three infiltrated bone and/or cartilage. All were amelanotic spindle cell tumors, five were neurotropic, and two were vasoinvasive. Aberrant p53 expression was seen in six tumors. All tumors recurred locally, and distant metastasis developed in three. Six patients died with disease (survival, 1-41 years; median, 8 years), and one patient was free of disease (survival 20 years). CONCLUSIONS: Primary mucosal desmoplastic melanomas are clinically characterized by local recurrence and may lead to distant metastasis and death even after several years. Alterations in p53 expression are frequently associated with desmoplastic melanoma.