Zhao Dan, Dai Jian, Hu Yu, Wang Tao
Department of Otorhinolaryngology-Head and Neck Surgery, The Lu An Hospital of Anhui Medical University, Lu An, Anhui, China.
Ear Nose Throat J. 2025 Jun;104(6):346-350. doi: 10.1177/01455613241276673. Epub 2024 Oct 8.
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.
低度纤维黏液样肉瘤（LGFMS）是一种极为罕见的软组织肿瘤，诊断颇具挑战性，且因术后极易复发和增殖而声名狼藉。LGFMS的主要症状包括鼻塞和流涕，并伴有面颊麻木和肿胀。在本文中，我们报告了一例起源于上颌窦（MS）的低度LGFMS的诊断和治疗情况。一名64岁男性被诊断为左上颌窦LGFMS，在1年的时间里接受了3次手术，随后出现局部复发。初次手术后术后病理结果不明确，患者术后2个月复发，需要进行第二次手术，该手术在病理上确诊为LGFMS。第二次手术后1个月开始放疗；然而，6个月后复发，导致进行第三次手术。随后，第三次手术后8个月再次复发，患者目前正在接受靶向治疗。通过讲述起源于上颌窦的LGFMS的诊断和病程，该病例突显了LGFMS固有的独特特征和治疗挑战。
