Korean J Dermatol.  2022 Jul;60(6):378-382.

A Case of Pleomorphic Dermal Sarcoma and Literature Review according to World Health Organization Reclassification Guideline

• Kim HH
• Myung KB
• Kwon ES
• Cheong SH

Abstract

Pleomorphic dermal sarcoma (PDS), formerly known as malignant fibrous histiocytoma (MFH), is a rare mesenchymal tissue tumor. A 40-year-old male presented with a 2-month history of a well-defined, solitary, round, palpable protruding mass with central ulceration, 1.5 cm in diameter. Punch biopsy showed tumor cells of variable size and hyperchromatic nuclei with frequent bizarre atypical cells and atypical mitoses arranged in a storiform-pleomorphic pattern in the entire dermis. After complete resection, PDS was diagnosed because the lesion had invaded the deep subcutaneous fat. Because MFH has been renamed according to the World Health Organization new classification system, we reclassified the 10 cases that have been reported as MFH in Korea. Nine cases were renamed undifferentiated pleomorphic sarcoma, with only one being renamed mucofibrosarcoma. Herein, we report a rare case of PDS treated with surgical excision with a review of the literature.

Keyword

Atypical fibroxanthoma; Malignant fibrous histiocytoma; Pleomorphic dermal sarcoma; Undifferentiated pleomorphic sarcoma