CASE REPORT article
Front. Oncol.
Sec. Cancer Immunity and Immunotherapy
This article is part of the Research TopicAdvancements in Solid Tumor Immunotherapy: Enhancing Efficacy and Overcoming ResistanceView all 9 articles
Sintilimab Combined with Chemotherapy in Advanced Pulmonary Epithelioid Hemangioendothelioma: A Case Report and Translational Insights
Provisionally accepted
- Weifang No.2 Peoplès Hospital, Weifang, China
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Background: Pulmonary epithelioid hemangioendothelioma (PEH) is an exceedingly rare vascular tumor, presenting a significant challenge due to its limited treatment options.Immunotherapy in combination with chemotherapy emerges as a potential frontier,yet the understanding of its application in PEH remains in its infancy. Methods:A male patient initially faced misdiagnosis as having aspergillosis.Through histopathology and immunohistochemistry,a definitive diagnosis of PEH was later established.The treatment journey involved surgical resection,followed by chemotherapy with albumin-bound paclitaxel and carboplatin,and finally immunotherapy with sintilimab. Results:A remarkable radiological improvement was observed post-sintilimab administration,leading to disease stabilization. Significantly,this is the first-ever report of the efficacy of sintilimab in PD-L1-high PEH,filling a critical gap in the existing literature. Conclusion:This case not only underscores the potential of sintilimab in PD-L1-high PEH but also sets a precedent for further exploration of immune checkpoint inhibitors in this rare disease. Translational Relevance: For the first time, we provide concrete evidence of sintilimab's activity in PD-L1-high PEH.This finding holds great promise,suggesting that immune checkpoint inhibitors could be a game-changer in the treatment of selected vascular sarcoma subtypes. Additionally,considering that about 90% of PEH cases harbor the characteristic WWTR1-CAMTA1 fusion gene,we hypothesize that this fusion might disrupt the normal immune-surveillance mechanism. It could potentially lead to the down-regulation of antigen-presenting machinery on tumor cells,making them less recognizable to the immune system. Alternatively,it may trigger the secretion of immunosuppressive cytokines in the tumor microenvironment,dampening the activation of immune effector cells.Further research is urgently needed to validate this hypothesis.
Keywords: Pulmonary epithelioid hemangioendothelioma, Sintilimab, PD-L1, Immunotherapy, WWTR1-CAMTA1 fusion
Received: 11 Feb 2025; Accepted: 07 Nov 2025.
Copyright: © 2025 Jing, An, Yang, Zhang, Li and Wang. 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: Yongming Wang, asdf2930374@sina.com
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