Crow-Fukase Syndrome Associated with Extramedullary Plasmacytoma

Abstract

A patient with extramedullary solitary tumor had progressive sensory motor neuropathy, generalized pigmentation of skin, pretibial edema and gynecomastia. Serological examination of this patient showed monoclonal IgA-λ gammopathy. Histologically, the tumor was defined as an IgA (λ type) positive extramedullary plasmacytoma by using peroxidase anti-peroxidase staining. The tumor was accompanied by angiofollicular lymphoid hyperplasia. Pathologically, it was recognized as an extramedullary plasmacytoma associated with Castleman's disease like changes, and was successfully treated by surgical resection, followed by irradiation.