Nasal Mucosal Desmoplastic Melanoma: A Case Report with Review of the Literature

Mucosal melanoma is a rare subtype of melanoma that accounts for 1% of all melanomas. The incidence of nasal mucosal melanomas is 0.3 per million. Desmoplastic melanomas are a subtype of melanoma with a reported incidence of 2.0 per million. Although 50% of desmoplastic melanomas are found in the head and neck region, mucosal desmoplastic melanoma is exceedingly rare. In the present study, we report a case of nasal mucosal desmoplastic melanoma and review the literature. A 79-year-old female presented to an outside otolaryngologist with nasal discomfort accompanied by rhinorrhea and was found to have a nasal vestibule mass. An endonasal incisional biopsy was performed yielding a diagnosis of a SOX-10 positive tumor. The patient was referred to our institution for further management. A blue-tinged lesion was identified at the prior biopsy site, and the mass was resected via an open rhinoplasty approach. Final pathology demonstrated an infiltrative spindle cell neoplasm with immunohistochemical patterns supportive of desmoplastic melanoma arising from the nasal vestibule. Due to positive margins, the patient underwent a re-resection with no tumor identified on the re-resected specimen. To our knowledge, this is the third case of nasal mucosal desmoplastic melanoma. We review the clinicopathologic features and management of this rare entity.