Giant Dermatofibrosarcoma Protuberans Presenting with Atypical Clinical Course / 대한피부과학회지

ABSTRACT

Dermatofibrosarcoma protuberans is a slow-growing malignant tumor of soft tissue usually occurring on the trunk or proximal extremities. It commonly affects 20 to 50-year-old persons and measures less than 5 cm in diameter. It starts as an erythematous indurated papule or plaque, and can enlarge over several years. It shows aggressive local invasion but a low rate of distant metastasis. A 63-year-old female presented with a 5-month-history of a rapidly growing cutaneous lesion on the abdomen. Physical examination revealed an 8x10 cm sized, violaceous, polypoid firm mass superimposed on the violaceous to brownish indurated plaque and surrounding nodule. Histopathologic examination showed an infiltrating mass composed of spindle shaped cells that were arranged in a storiform growth pattern within the whole layer of dermis. The cellular mass extended into the subcutaneous fat layer in a honeycomb pattern. The tumor cells were positive for CD-34 stain. Additionally, some areas of tumor showed myxoid change. Magnetic resonance imaging demonstrated a homogenously-enhancing, and protruded mass invading the subcutaneous fat layer. The diagnosis of dermatofibrosarcoma protuberans was established and it was treated by wide local excision. We report a case of giant, polypoid dermatofibrosarcoma protuberans presenting with rapid growth.