Biology, classification, and management of recurrent myxofibrosarcoma 21 years after resection

Soft-tissue sarcomas (STSs) are a heterogenous group of rare malignancies that have significant lifelong implications. Accepted management options include limb-sparing surgical resection and adjuvant radiation therapy. Here we present the case of a myxoid malignant fibrous histiocytoma, now termed a myxofibrosarcoma, which recurred 21 years after primary surgical resection. To our knowledge, this is the longest documented interval between initial management and recurrence of an STS. Significant changes have been made in classification guidelines and diagnostic methods over this 2-decade period. The pathogenesis of remote recurrence of STSs remains controversial and is discussed in this report.