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.