AUTHOR=Jing Lijun , An Huiyun , Yang Hao , Zhang Liming , Li Tongyuan , Wang Yongming 

TITLE=Sintilimab combined with chemotherapy in advanced pulmonary epithelioid hemangioendothelioma: a case report and translational insights

JOURNAL=Frontiers in Oncology

VOLUME=Volume 15 - 2025

YEAR=2025

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2025.1575039

DOI=10.3389/fonc.2025.1575039

ISSN=2234-943X

ABSTRACT=BackgroundPulmonary 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.MethodsA 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.ResultsA 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.ConclusionThis 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.