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

Frontiers in oncology 2025 Vol.15() p. 1575039

Jing L, An H, Yang H, Zhang L, Li T, Wang Y

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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.

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APA Jing L, An H, et al. (2025). Sintilimab combined with chemotherapy in advanced pulmonary epithelioid hemangioendothelioma: a case report and translational insights.. Frontiers in oncology, 15, 1575039. https://doi.org/10.3389/fonc.2025.1575039
MLA Jing L, et al.. "Sintilimab combined with chemotherapy in advanced pulmonary epithelioid hemangioendothelioma: a case report and translational insights.." Frontiers in oncology, vol. 15, 2025, pp. 1575039.
PMID 41357594

Abstract

[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.

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