Current Discoveries and Management of Psammomatous Melanotic Schwannoma

Psammomatous melanotic schwannoma is a rare form of schwannoma that is distinct in terms of presentation, location, and nature compared to other types. This tumor is highly associated with Carney complex, but has also been seen in the neurofibromatosis syndromes. Although discovered over 80 years ago, official management guidelines have not been established. The following review comprises the current understanding of this rare tumor including genetic associations, presentation, imaging, pathology, prognosis, and management.