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KMID : 0363220100480040318
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Korean Journal of Dermatology
2010 Volume.48 No. 4 p.318 ~ p.321
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Giant Dermatofibrosarcoma Protuberans Presenting with Atypical Clinical Course
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Choi Chang-Min
Lew Bark-Lynn
Sim Woo-Young
Cho Hee-Ryung
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Abstract
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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 8×10 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.
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KEYWORD
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Dermatofibrosarcoma protuberance, Giant, Myxoid
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