Psammomatous melanotic schwannoma as a component of Carney complex

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 difficult 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 findings with immunohistochemistry correlation a final diagnosis of psammomatous melanotic schwannoma was rendered.