Naik Vinayak Gourish, Rai Kirthi Kumar, Shivakumar H R
Department of Oral and Maxillofacial Surgery, Bapuji Dental College and Hospital, Davangere, Karnataka, India.
Natl J Maxillofac Surg. 2021 May-Aug;12(2):271-275. doi: 10.4103/njms.NJMS_54_15. Epub 2021 Jul 15.
Low-grade fibromyxoid sarcoma (LGFMS) is a rare type of sarcoma that is characterized by benign-appearing histologic features but a paradoxically aggressive clinical course. Recognition of this lesion is important because of its indolent but metastasizing nature. These tumors generally occur in young to middle-aged adults, sometimes in children, but rarely in the high-aged adults. LGFMS typically affects the deep soft tissues of the trunk or lower extremities: however, it is rarely seen on the maxillofacial region. Here, we describe a case of LGFMS on the left lower one-third region of the face of a 35-year-old male patient with a 6-month history. On gross examination, the resected specimen consisted of an open ovoid mass of 2 cm × 2 cm × 1 cm. Light microscopy revealed well-circumscribed myxoid tumor with hypocellular areas in nodules merging to collagenized areas. Immunohistochemical examination revealed diffuse positivity to vimentin, whereas tests for desmin, S-100 protein were negative, thus confirming the diagnosis. After the initial healing of the surgical wound, the patient was advised 30 cycles of radiotherapy. Recurrence and metastasis have not been observed for 1 year of surgical excision now. Due to the notably indolent nature of LGFMS, long-term follow-up is necessary to evaluate its clinical course.
低度纤维黏液样肉瘤（LGFMS）是一种罕见的肉瘤，其组织学特征看似良性，但临床病程却具有矛盾的侵袭性。认识这种病变很重要，因为它具有惰性但会转移的特性。这些肿瘤通常发生在年轻至中年成年人身上，有时也见于儿童，但很少发生在高龄成年人身上。LGFMS通常累及躯干或下肢的深部软组织；然而，在颌面区域很少见。在此，我们描述一例35岁男性患者，其左脸下三分之一区域出现LGFMS，病程6个月。大体检查时，切除标本为一个2 cm×2 cm×1 cm的开放卵圆形肿块。光镜检查显示为边界清楚的黏液样肿瘤，结节内细胞稀少区域与胶原化区域融合。免疫组化检查显示波形蛋白弥漫阳性，而结蛋白、S-100蛋白检测为阴性，从而确诊。手术伤口初步愈合后，建议患者进行30个周期的放疗。手术切除至今1年，未观察到复发和转移。由于LGFMS具有明显的惰性，需要长期随访以评估其临床病程。
