Abstract
Low-grade myofibroblastic sarcoma (LGMS) is an extremely rare mesenchymal neoplasm. It arises from the differentiation of myofibroblasts and demonstrates a preference for the head and neck region. Herein, we present a case of LGMS in a 52-year-old man with painless swelling in the anterior right area of the tongue. Histologically, the lesion consisted of spindle-shaped cells exhibiting mild to moderate nuclear atypia, arranged in fascicles with a storiform pattern. Neoplastic cells were positive for SMA and negative for EMA, CD34, h-caldesmon, desmin, β-catenin, S100, SOX10, and NF, with a Ki-67 index of 15-20%. Complete surgical excision with clear margins was the treatment of choice. Very few cases of LGMS have been documented in the literature, and regular follow-up for these patients is essential for drawing reliable conclusions regarding local recurrence and the metastatic potential of this tumor. Key words:Low-grade, myofibroblastic, sarcoma, tongue, myofibroblasts, immunohistochemistry.