Related Experiment Video
Updated: Jul 4, 2025
Author Spotlight: Anterior HR-OCT as a Non-Invasive Tool for Characterizing Ocular Surface Squamous Neoplasia
Published on: August 9, 2024
Sclerosing Epithelioid Fibrosarcoma
Laura M Warmke1, Wendong Yu2, Jeanne M Meis2
1Department of Pathology and Laboratory Medicine, Indiana University, IU Health Pathology Laboratory, 350 W 11th Street, Room 4086, Indianapolis, IN 46202, USA.
Abstract:
Sclerosing epithelioid fibrosarcoma (SEF) is a distinctive sarcoma that may arise in nearly any soft tissue site or bone. While there has been past controversy as to whether it is related to low-grade fibromyxoid sarcoma (LGFMS), it has been shown to behave far more aggressively than LGFMS. SEF has a propensity to metastasize to the lungs and bone and arise within the abdominal cavity. Histologically, it is characterized by uniform nuclei embedded in a densely collagenous stroma simulating osteoid. By immunohistochemistry, it is often strongly positive for MUC4. The majority (75%) have EWSR1 gene rearrangement, most commonly with CREB3L1 as a fusion partner, although a variety of FUS/EWSR1 and CREB3L1/CREB3L2/CREB3L3 fusions have been described in addition to others. SEF is currently recalcitrant to nearly all chemotherapy and radiation therapy.
