Abstract
Low-grade myofibroblastic sarcoma (LGMS) is categorized as an extremely rare malignant neoplasia of myofibroblasts. To date, only 38 cases describing patients diagnosed with LGMS in the oro-maxillofacial region have been reported in the scientific literature. Here, we introduce a rare case report and, for the first time, also provide a recent update of the literature and a clear review regarding the immunohistochemical panel to diagnose this entity.