Dermatofibrosarcoma protuberans metastatic to the orbit.

A61-YEAR-OLD MAN had pain, redness, and decreased motilityoftherighteye of 2 months’ duration. He had undergone excision of alumbardermatofibrosarcomaprotuberans(DFSP)2yearsearlier.His visual acuity was hand motions in the right eye. Examination showed proptosisandseveremotilityrestriction. Computed tomography (Figure 1) and needle biopsy substantiated the suspicion of metastatic DFSP to the orbit. The patient underwent orbital radiation therapy to reduce pain symptoms. Palliative chemotherapy was given for his multiple concurrent systemic metastases. Nine months after initial examination, the patient requestedsurgicalmanagementbecause of continued discomfort and worsening disfigurement of his orbit (Figure 2). Aftercarefulconsiderationofthe patient’soverallhealth,apalliativelidsparingorbitalexenterationwasperformed.Histopathologicevaluationof the specimen showed spindleshaped fibroblasts in a hypercellular storiformpattern(Figure3),andthe tumor cells were positive for CD34. Somepleomorphismandmultinucleated giant cells were present, characteristic of a more malignant variant of DFSP. 1 Comparison with the histopathologic features of the patient’sprimarylumbarDFSPshowed findings consistent with metastasis. Three days after surgery, the patient hadmildpostoperativeeyelidedema (Figure 4) and reported a substantial improvement in symptoms. Six monthsaftersurgery,thepatientcontinued to report stable symptoms. COMMENT