Myxofibrosarcoma: Outcomes, Prognostic Factors, and Role of Neoadjuvant Radiation Therapy

Abstract

IntroductionMyxofibrosarcoma (MFS) is a subtype of soft tissue sarcoma with a highly infiltrative growth pattern that leads to a higher risk of inadvertent positive surgical margins and local relapse. Poorly defined tumor margins also pose a challenge for radiotherapy (RT) planning, in terms of treatment volumes and administration of pre- versus post-operative RT. This study aims to evaluate local control and patterns of recurrence in MFS patients treated with neoadjuvant RT followed by definitive surgical excision. Materials and methodsMultiple institutional databases were retrospectively searched for patients diagnosed with MFS between 2013-2021, who were exclusively treated with pre-operative radiotherapy followed by definitive surgery at our institution. The endpoints of the study were defined as local tumor recurrence (LR), distant metastasis (DM), or death from the date of definitive surgery. Results49 patients met the inclusion criteria and were included in the final study. The median age at diagnosis was 67 years, and 71% were male. The tumor was superficially located in 63% of patients and the mean tumor size at presentation was 7.8 cm. All patients received neoadjuvant RT and completed their planned course of treatment. Neoadjuvant chemotherapy was administered in 22% of patients. Inadvertent excision (IE) prior to definitive treatment was performed in 25 patients (51%), 84% of which had superficially located tumors.All margins were assessed using frozen section analysis at the time of definitive surgery, and 100% of patients had negative surgical margins, with 25% having no residual tumor. With a median follow-up of 4.7 years, a 5-year local control (LC) rate was 87%, and 5-year overall survival (OS) was 98%. Tumor depth was associated with DM (p <0.01). ConclusionDespite the infiltrative nature of MFS, preoperative RT followed by definitive surgical excision, especially in the setting of a reliable frozen section margin analysis, is associated with excellent local control.