Abstract：
Low-Grade Myofibroblastic sarcoma (LGMS) is categorized as an extremely rare malignant neoplasia of myofibroblasts, which has only recently become more widely studied. Our patient was referred for evaluation of a nodule involving the palate. The histopathological analysis showed a mesenchymal tumor formed for stellate-shaped cells. Negativity for Laminin, Desmin, Collagen IV, CK pool, CD34, S100, and ALK1, discarded epithelial, endothelial, neural, and inflammatory origin. On the other hand, the positivity for Calponin and SMA demonstrated myofibroblastic and smooth muscle differentiation. The diagnosis of LGMS was endorsed and the patient was submitted for medical treatment. To date, only 18 cases describing patients diagnosed with intrabuccal LGMS have been reported in the scientific literature. Here, we introduce a rare report and for the first time, also provide an update of the literature and a clear review regarding the immunohistochemical panel to diagnose this entity, once the histopathological diagnosis is still challenging.
摘要：
低度肌纤维母细胞肉瘤（LGMS）被归类为肌纤维母细胞的极其罕见的恶性肿瘤，直到最近才得到更广泛的研究。我们的患者被转诊以评估涉及腭的结节。组织病理学分析显示，星形细胞形成了间质肿瘤。层粘连蛋白的否定，Desmin,胶原蛋白IV,CK游泳池,CD34，S100和ALK1，丢弃上皮，内皮，神经,和炎症起源。另一方面,Calponin和SMA阳性显示肌纤维母细胞和平滑肌分化。LGMS的诊断得到认可，患者接受治疗。迄今为止,在科学文献中,仅有18例描述被诊断为颊内LGMS的患者.这里,我们介绍了一份罕见的报告，这是第一次，还提供了文献的更新和关于免疫组织化学小组的明确审查，以诊断该实体，一旦组织病理学诊断仍然具有挑战性。
