Sclerosing Epithelioid Fibrosarcoma - 24/01/24

Doi : 10.1016/j.path.2023.06.009

Laura M. Warmke, MD ^a, Wendong Yu, MD, PhD ^b, Jeanne M. Meis, MD ^b,^⁎
^a Department of Pathology and Laboratory Medicine, Indiana University, IU Health Pathology Laboratory, 350 W 11th Street, Room 4086, Indianapolis, IN 46202, USA
^b Department of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Pathology Unit #085, Houston, TX 77030, USA

^∗Corresponding author. Department of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Un #085, 1515 Holcombe Boulevard, Houston, TX 77005.Department of Pathology and Laboratory MedicineThe University of Texas MD Anderson Cancer CenterUn #0851515 Holcombe BoulevardHoustonTX77005

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.

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Keywords : Sclerosing epithelioid fibrosarcoma, Low-grade fibromyxoid sarcoma, MUC4, EWSR1, FUS, CREB3L1, CREB3L2, KMT2A