CASE REPORT article
Front. Oncol.
Sec. Pediatric Oncology
Volume 15 - 2025 | doi: 10.3389/fonc.2025.1613906
This article is part of the Research TopicRefractory and Relapsed Hemophagocytic Lymphohistiocytosis in Pediatric Population: Targeted TherapyView all 4 articles
Uncommon Presentation of Ewing Sarcoma Complicated by Hemophagocytic Lymphohistiocytosis: A Diagnostic Dilemma and Therapeutic Challenge
Provisionally accepted
- 1Department of Emergency, West China Second University Hospital, Sichuan University, Chengdu, China, Chengdu, Sichuan Province, China
- 2Key Laboratory of Birth Defects and Related Diseases of Women and Children, West China Second University Hospital, Sichuan University, Chengdu, Sichuan Province, China
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Hemophagocytic lymphohistiocytosis (HLH), also referred to as hemophagocytic syndrome (HPS), is a life-threatening condition characterized by excessive immune activation. It is commonly associated with genetic mutations, infections, autoimmune diseases, and malignancies.Malignancy-associated HLH (M-HLH) is most frequently observed in hematologic malignancies, such as lymphoma and leukemia, while its occurrence in solid tumors is exceedingly rare. Here, we report a unique case of temporal bone Ewing sarcoma complicated by HLH and Epstein-Barr virus (EBV) infection. Despite intensive chemotherapy, the patient ultimately succumbed to multi-organ failure and septic shock. To the best of our knowledge, this is the first documented case of Ewing sarcoma associated with HLH.
Keywords: hemophagocytic lymphohistiocytosis, Extraosseous ewing sarcoma, case report, Epstein-Barr virus ( EBV), malignancy-associated HLH
Received: 02 May 2025; Accepted: 21 Jul 2025.
Copyright: © 2025 Zou, Chen, Tang, Li and Huang. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Chengshuang Huang, Department of Emergency, West China Second University Hospital, Sichuan University, Chengdu, China, Chengdu, Sichuan Province, China
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