Surgical Management of Low-Grade Fibromyxoid Sarcoma in the Otolaryngologic Region: A Report of 11 Cases and Literature Review

Background: Low-grade fibromyxoid sarcoma (LGFMS) in the otorhinolaryngologic region remains exceptionally rare, with limited reports in the literature. The aim of this study was to report 11 cases of otorhinolaryngologic LGFMS and to review the literature with respect to the epidemiology, clinical presentation, diagnosis, treatment, and prognosis of this disease. Methods: We conducted a retrospective analysis of 11 patients referred to our hospital over a 10-year period (2015– 2025). Results: The cohort comprised five male and six female patients. The primary sites were distributed across multiple anatomical locations including the sphenoid sinus, nasopharynx, nasal cavity, vocal cord, maxillary sinus, and ethmoid sinus. The presenting symptoms were nonspecific. All patients underwent suspension laryngoscopy, endoscopic resection, combined endoscopic and open resection, or open approaches. All three patients with positive margins experienced recurrence during follow-up (at 4, 4, and 13 months, respectively), whereas none of the eight patients with negative margins has experienced recurrence to date (median follow-up: 94 months, range 82– 112 months). Furthermore, only two patients who died had positive surgical margins. Upon initial diagnosis, 7 of 11 cases presented with T2-stage disease or higher. After a median follow-up of 89 months (range, 4– 112), Kaplan-Meier analysis of all 11 patients revealed 3- and 5-year overall survival(OS) rates of 90.0% each, with corresponding progression-free survival(PFS) rates of 72.7% at both time points. Patients with incomplete follow-up were included as censored observations (1 lost to follow-up, 1 non-disease-related death). Conclusion: LGFMS of the otorhinolaryngologic region is a rare neoplasm with diverse primary sites, for which surgical resection remains the primary treatment. In our series, the surgical approach—suspension laryngoscopy, endoscopic resection, combined endoscopic and open resection, or open approaches—was selected on a case-by-case basis according to tumor location and extent, with the aim of achieving adequate surgical margins. No single approach emerged as a preferred or standardized strategy from this small retrospective series; rather, these observations reflect individualized surgical decision-making. Re-excision to achieve negative margins may be considered, but evidence supporting this approach remains limited, particularly in the otorhinolaryngologic region. These observations should be interpreted with caution given the limited sample size, and both tumor stage and resection margin status appear to be potential prognostic factors that require validation in larger studies. The efficacy of adjuvant radiotherapy remains inconclusive, given mixed outcomes reported in the literature. Improving patient compliance with prolonged surveillance is a critical component of management. This study is subject to limitations including its retrospective design, single-centre setting, and small cohort size (n=11).