Coexistent Orbital Dermatofibrosarcoma Protuberans and Bilateral Lymphoid Hyperplasia

Abstract

A 72-year-old man had secondary orbital involvement with dermatofibrosarcoma protuberans 36 years after removal of the initial tumor on his forehead. In addition, multiple bilateral inferior orbital masses were present, which on pathologic examination proved to be reactive lymphoid hyperplasia. Serum immunoelectrophoresis revealed polyclonal elevations of IgG and IgA. Dermatofibrosarcoma protuberans is part of the spectrum of fibrohistiocytic tumors that also includes atypical fibroxanthoma and benign and malignant fibrous histiocytoma. The exact cell of origin of dermatofibrosarcoma protuberans is controversial, though immunohistochemical study of our specimen supports the fibroblast. The coexistence of this rare orbital tumor with noncontiguous reactive lymphoid hyperplasia is unique, and points out the fact that multiple orbital masses may indicate more than one underlying disease process.