Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge
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...
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Frontiers Media S.A.
2025-08-01
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| Series: | Frontiers in Oncology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fonc.2025.1613906/full |
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| author | Zhuan Zou Zhuan Zou Bin Chen Bin Chen Fajuan Tang Fajuan Tang Xihong Li Xihong Li Chengshuang Huang Chengshuang Huang |
| author_facet | Zhuan Zou Zhuan Zou Bin Chen Bin Chen Fajuan Tang Fajuan Tang Xihong Li Xihong Li Chengshuang Huang Chengshuang Huang |
| author_sort | Zhuan Zou |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-e9fd7ac569cd4218870a21b9e5ea7ae6 |
| institution | Kabale University |
| issn | 2234-943X |
| language | English |
| publishDate | 2025-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Oncology |
| spelling | doaj-art-e9fd7ac569cd4218870a21b9e5ea7ae62025-08-20T03:59:45ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2025-08-011510.3389/fonc.2025.16139061613906Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challengeZhuan Zou0Zhuan Zou1Bin Chen2Bin Chen3Fajuan Tang4Fajuan Tang5Xihong Li6Xihong Li7Chengshuang Huang8Chengshuang Huang9Department of Emergency, West China Second University Hospital, Sichuan University, Chengdu, ChinaKey Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, ChinaDepartment of Emergency, West China Second University Hospital, Sichuan University, Chengdu, ChinaKey Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, ChinaDepartment of Emergency, West China Second University Hospital, Sichuan University, Chengdu, ChinaKey Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, ChinaDepartment of Emergency, West China Second University Hospital, Sichuan University, Chengdu, ChinaKey Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, ChinaDepartment of Emergency, West China Second University Hospital, Sichuan University, Chengdu, ChinaKey Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, ChinaHemophagocytic 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.https://www.frontiersin.org/articles/10.3389/fonc.2025.1613906/fullhemophagocytic lymphohistiocytosisextraosseous ewing sarcomacase reportEpstein-Barr virus (EBV)malignancy-associated HLH |
| spellingShingle | Zhuan Zou Zhuan Zou Bin Chen Bin Chen Fajuan Tang Fajuan Tang Xihong Li Xihong Li Chengshuang Huang Chengshuang Huang Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge Frontiers in Oncology hemophagocytic lymphohistiocytosis extraosseous ewing sarcoma case report Epstein-Barr virus (EBV) malignancy-associated HLH |
| title | Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge |
| title_full | Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge |
| title_fullStr | Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge |
| title_full_unstemmed | Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge |
| title_short | Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge |
| title_sort | case report uncommon presentation of ewing sarcoma complicated by hemophagocytic lymphohistiocytosis a diagnostic dilemma and therapeutic challenge |
| topic | hemophagocytic lymphohistiocytosis extraosseous ewing sarcoma case report Epstein-Barr virus (EBV) malignancy-associated HLH |
| url | https://www.frontiersin.org/articles/10.3389/fonc.2025.1613906/full |
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