Abstract
Low-grade fibromyxoid sarcoma (LGFMS) represents an exceptionally rare soft-tissue tumor, challenging to diagnose, and notorious for relentless recurrence and proliferation postsurgical resection. Primary symptoms of LGFMS include nasal congestion and rhinorrhea, accompanied by cheek numbness and distension. In this article, we report the diagnosis and treatment of a case of low-grade LGFMS originating in the maxillary sinus (MS). A 64-year-old male diagnosed with LGFMS of the left MS, undergoing 3 surgeries over a 1-year period with subsequent local recurrence. Following inconclusive postoperative pathology after the initial surgery, the patient experienced recurrence 2 months postsurgery, necessitating a second operation, which confirmed the LGFMS diagnosis pathologically. Radiation therapy commenced 1 month after the second surgery; however, recurrence transpired 6 months later, leading to a third operation. Subsequently, recurrence occurred again 8 months post third surgery, with the patient currently undergoing targeted therapy. This case underscores the distinct characteristics and therapeutic challenges inherent in LGFMS through the narrative of diagnosis and progression of LGFMS originating in the MS.
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