Primary low-grade myofibroblastic sarcoma: A rare case report of this tumor in the orbit and literature review

Abstract

Low-grade myofibroblastic sarcoma (LGMS) is an exceedingly rare, malignant tumor with myofibroblastic differentiation. It frequently occurs in the oral cavity and extremities, despite being classified as a distinct entity by the World Health Organization (WHO). Here, we report a rare case of orbital LGMS occurring in an 11-month-old baby with a 3.2 × 2.4 × 2.1 cm mass. LGMS was diagnosed based on the histological and immunohistochemical findings. Previous literature suggests that surgical resection with clear margins is an appropriate method for the treatment of LGMS, and combined adjuvant therapy (local radiotherapy with or without chemotherapy) can improve the prognosis, but further studies are needed.