Psammomatous melanotic schwannoma as a component of Carney complex.

ABSTRACT

Melanotic schwannoma is a rare form of pigmented neural tumor commonly arising from the posterior spinal nerves and ganglia. Two variants have been described, psammomatous and nonpsammomatous. 50% of psammomatous tumors are associated with Carney complex. The biologic behavior of the tumor is diffi cult to predict and slightly over 10% of the tumors follow malignant course. We present a case of psammomatous melanotic schwannoma as part of Carney complex in a 67-year-old male. Clinical examination revealed oral mucosal and abdominal skin pigmentation. Magnetic resonance imaging showed an intradural extramedullary lesion at D8-D12 level. Intraoperative squash smear study showed sheets of spindle cells with abundant intracytoplasmic melanin pigmentation and few psammoma bodies. Based on clinical, radiologic, and histopathological fi ndings with immunohistochemistry correlation a fi nal diagnosis of psammomatous melanotic schwannoma was rendered.