Dermatology Online Journal

Pleomorphic dermal sarcoma is a cutaneous soft tissue sarcoma that presents as a rapidly enlarging tumor, typically on a sun-exposed location of elderly individuals. The neoplasm shares many similar features — clinical, pathologic, immunohistochemical and genomic — with atypical fibroxanthoma. However, adverse histologic characteristics (deep subcutaneous invasion, tumor necrosis, lymphovascular invasion, and/or perineural invasion) differentiate pleomorphic dermal sarcoma from atypical fibroxanthoma and may account for the more aggressive biologic behavior of pleomorphic dermal sarcoma: local recurrence and metastases. The features of a woman with pleomorphic dermal sarcoma are described. Her sarcoma presented as a rapidly growing ulcerated red nodule on the left side of her face. Imaging studies were performed prior to surgery. The tumor was extirpated with a wide local excision and she received postoperative radiotherapy. There has been no recurrence or metastasis at one-year follow-up. Pleomorphic dermal sarcoma has previously been referred to as a malignant fibrous histiocytoma (until the term became antiquated) and an undifferentiated pleomorphic sarcoma. However, the latter term includes not only neoplasms from the skin but also sarcomas from internal organs, retroperitoneal and osteoid origin. Therefore, when classifying this undifferentiated soft tissue sarcomas of cutaneous origin, the term pleomorphic dermal sarcoma may be preferred.