{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,7]],"date-time":"2026-05-07T15:52:53Z","timestamp":1778169173660,"version":"3.51.4"},"reference-count":28,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T00:00:00Z","timestamp":1754870400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3389\/fonc.2025.1613906","type":"journal-article","created":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T05:24:01Z","timestamp":1754889841000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge"],"prefix":"10.3389","volume":"15","author":[{"given":"Zhuan","family":"Zou","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bin","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fajuan","family":"Tang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xihong","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chengshuang","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,8,11]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"101515","DOI":"10.1016\/j.berh.2020.101515","article-title":"Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy","volume":"34","author":"Griffin","year":"2020","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.5858\/arpa.2020-0802-RA","article-title":"Hemophagocytic lymphohistiocytosis","volume":"146","author":"Ponnatt","year":"2022","journal-title":"Arch Pathol Lab Med"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1016\/j.hoc.2015.06.009","article-title":"Hemophagocytic lymphohistiocytosis in adults","volume":"29","author":"Campo","year":"2015","journal-title":"Hematol Oncol Clin North Am"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2023.1010561","article-title":"Hemophagocytic lymphohistiocytosis in gastric cancer: A rare syndrome for the oncologist. 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