Low-Grade Fibromyxoid Sarcoma Featuring an Unusual EWSR1-CREB3L2 Gene Fusion: Report of a Rare Case Arising in the Parotid Gland

Abstract

AbstractLow-grade fibromyxoid sarcoma (LGFMS), also known as Evan's tumor, is a fibroblastic tumor with a deceptively bland morphology but a high metastasizing potential and late recurrence. It presents clinically as a slowly growing mass in the deep soft tissue of extremities and trunk. Morphologically, it shows a biphasic pattern with alternating fibrous and myxoid areas, whorling growth pattern, hypocellularity, and bland fibroblasts with curvilinear or arcuate vessels in between. Collagen rosettes with central hyalinization is a helpful feature but not specific. MUC-4 is the most sensitive immunostain in LGFMS. The majority of cases harbor a gene fusion in FUS-CREB3L2. Herein, we present a 23-year-old female patient with a slow growing painless mass in the left parotid gland, which was later diagnosed as LGFMS. Involvement of the head and neck region by LGFMS is uncommonly encountered. To date, only two cases within the parotid gland have been described.