Despite recent clinical advances in treatment, sinonasal mucosal melanoma (SMM), a rare but aggressive disease, has a markedly poor prognosis. Unlike their cutaneous counterparts, neuroectodermal melanocytes residing in the respiratory mucosa, nasal stroma, and secretory glands serve as the major sites of malignant transformation, giving rise to tumors that together account for 1% to 2% of all melanomas.
1 Risk factors remain unclear, although an association with preexisting mucosal melanosis, formaldehyde exposure, and African or Japanese ancestry has been observed.
2-5 Given its proclivity for distant metastatic disease and local recurrence, 5-year survival rates for SMM have generally remained less than 35% as the overall worldwide incidence increases.
6-9 Interestingly, although the intrinsic behavior of melanoma arising from the squamous mucosa of the oral cavity and the respiratory mucosa of the sinonasal tract differs (the latter demonstrating a lower risk of nodal disease but higher rate of perineural invasion), overall prognosis for both forms remains comparable.
10,11