Low-grade myxofibrosarcoma presenting at the site of prior high-grade disease

Myxofibrosarcoma is one of the most common soft tissue sarcomas occurring in older adults. It can arise de novo or can be radiation induced, and the term myxofibrosarcoma was originally devised to encompass a spectrum of myxoid tumors with characteristics similar to malignant fibrous histiocytoma (MFH). Confusion exists, however, regarding the distinction between microscopic grade and characteristics of myxofibrosarcoma and MFH. Correct classification is vital to prognosis, as the degree of myxoid change is inversely related to the incidence of metastasis. We present a case of a 76-year-old man with a history of high-grade MFH of the left lower extremity, status post excision and radiation therapy, who presented 2 years later with a regional metastatic recurrence of high-grade MFH to the left groin as well as new nodules adjacent to and within his prior excision and radiation site. These new nodules were determined to represent low-grade myxofibrosarcoma. These new low-grade lesions either represent a low-grade recurrence of high-grade sarcoma or a new, radiation-induced soft tissue sarcoma occurring at the same site. Radiotherapy, however, is an unlikely cause; specific postradiation sarcoma criteria have not been fulfilled. This article discusses both the nosology and histopathological spectrum of these important soft tissue sarcomas, their aggressive and recurrent nature and their association with radiation therapy.