Abstract

Low-grade myofibroblastic sarcoma is a malignant mesenchymal tumor with myofibroblastic differentiation, characterized by a high rate of local recurrence but low metastatic potential. It is commonly identified in the head and neck region, and particularly in the oral cavity. We report the diagnostic and treatment steps of a fast-growing lesion of the lower left jaw in a 22-year-old otherwise healthy woman. Based on histological and immunohistochemical features, the diagnosis of low-grade myofibroblastic sarcoma was determined. After a careful evaluation and given the absence of established protocols, our multidisciplinary tumor board recommended segmental mandibulectomy combined with a selective neck dissection. Histopathological examination of the resected specimen confirmed a low-grade mesenchymal neoplasm with focal intermediate-grade areas, resected within oncologically safe margins. In view of the histopathological findings, the case was further discussed by the multidisciplinary tumor board, which recommended adjuvant radiotherapy. No evidence of local recurrence or metastatic disease has been observed during the 10-month postoperative follow-up. Given the rarity of this tumor and the limited number of reported cases, this case report provides further insight into its diagnosis and management and contributes to the existing evidence available for the treatment of this pathology.

IPC Classification

Keywords