Low-grade fibromyxoid sarcoma of the orbit

Angela J Oh; Pallavi Singh; Nathan Pirakitikulr; Kelsey Roelofs; Ben J Glasgow; Daniel B Rootman

doi:10.1080/01676830.2022.2149820

Low-grade fibromyxoid sarcoma of the orbit

Orbit. 2022 Nov 28:1-5. doi: 10.1080/01676830.2022.2149820. Online ahead of print.

Authors

Angela J Oh¹, Pallavi Singh¹, Nathan Pirakitikulr¹, Kelsey Roelofs¹, Ben J Glasgow², Daniel B Rootman¹

Affiliations

¹ Division of Orbital and Ophthalmic Plastic Surgery, Jules Stein Eye Institute, University of California, Los Angeles, California, USA.
² Division of Ophthalmic Pathology, Jules Stein Eye Institute, and Department of Pathology and Laboratory Medicine, University of California, Los Angeles, California, USA.

PMID: 36437749
DOI: 10.1080/01676830.2022.2149820

Abstract

A 70-year-old male presented with diplopia and painless proptosis of the left eye for 5 months. Examination showed 6 mm of axial proptosis and restriction of supraduction, abduction and adduction, and mild limitation of infraduction of the left eye. Magnetic resonance imaging demonstrated a large, moderately well-circumscribed intraconal mass in the left lateral orbit, and excisional biopsy was performed. Histopathologic features of mixed fibrous and myxoid areas in a whorl-like pattern and immunohistochemical staining for MUC4 confirmed the diagnosis of low-grade fibromyxoid sarcoma (LGFMS). Next-generation sequencing revealed genetic fusion of EWSR1-CREB3L1. LGFMS is an extremely rare neoplasm with only two prior documented cases of orbital involvement. Here, we report the third case of orbital LGFMS.

Keywords: Fibromyxoid; orbit; sarcoma.