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CC BY-NC-ND 4.0 · Avicenna J Med 2022; 12(02): 087-092
DOI: 10.1055/s-0042-1749611
Case Report

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

Doaa M. Alayed
1   Department of Pathology and Laboratory Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia
,
Majed M. Pharaon 
2   Department of Pathology and Laboratory Medicine, Ministry of National Guard, Riyadh, Saudi Arabia
3   King Abdullah International Medical Research Center, Ministry of National Guard, Riyadh, Saudi Arabia
› Author Affiliations Funding None.
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Abstract

Low-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.

Keywords

low-grade fibromyxoid sarcoma - Evan's tumor - parotid gland - MUC-4

Availability of Data and Material

All data regarding this case is available at our department as actual slides or digitally as photos and soft copies.


Consent for Publication

All authors read and approved the manuscript.


Ethical Approval

All study procedures were performed in accordance with the Declarations of Helsinki, 1964.




Publication History

Article published online:
21 June 2022

© 2022. Syrian American Medical Society. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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