Pulmonary epithelioid hemangioendothelioma with pleural dissemination: a case report of prolonged diagnostic delay and the role of surgical biopsy.
5 affiliations
- 1Department of Critical Care Medicine, Huashan Hospital, Fudan University, Shanghai, China.
- 2Pathology Department, Huashan Hospital, Fudan University, Shanghai, China.
- 3Department of Respiratory Diseases and Critical Medicine, Huashan Hospital, Fudan University , Shanghai, China. [email protected].
- 4Department of Respiratory Diseases and Critical Medicine, Huashan Hospital, Fudan University , Shanghai, China. [email protected].
- 5Department of Respiratory Diseases and Critical Medicine, Quzhou Hospital, Wenzhou Medical University, Quzhou, Zhejiang, 324000, China. [email protected].
Abstract
Background
Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor with low incidence and nonspecific clinical and radiological features, frequently leading to misdiagnosis and delayed diagnosis.Case presentation
We report a 32-year-old man with bilateral pulmonary nodules that were followed for 6 years before the development of a new left lower lobe mass, pleuritic chest pain, dyspnea, and massive pleural effusion in 2025. Initial imaging revealed diffuse miliary nodules that remained relatively indolent for several years. Two transbronchial lung biopsies (TBLB) and repeated pleural fluid cytology failed to establish a diagnosis. Definitive diagnosis was achieved only after video-assisted thoracoscopic surgery (VATS), with immunohistochemical support for endothelial differentiation and molecular confirmation of the WWTR1-CAMTA1 fusion gene. The patient underwent surgery, systemic therapy, and intrapleural treatment, followed by maintenance sirolimus. Disease remained clinically and radiologically stable during 6 months of follow-up.Conclusions
PEH presents substantial diagnostic challenges because of its indolent course and nonspecific clinical and radiological features. For patients with unexplained multiple pulmonary nodules and pleural involvement, especially when minimally invasive investigations remain repeatedly nondiagnostic, early surgical biopsy combined with endothelial immunophenotyping, and molecular testing is essential for timely diagnosis and optimal management.
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BibTeX
@article{yan2026pulmonary,
title = {{Pulmonary epithelioid hemangioendothelioma with pleural dissemination: a case report of prolonged diagnostic delay and the role of surgical biopsy.}},
author = {Rui Yan and Weiwei Feng and Lu Lu and Yi Gong and Guifang Wang},
journal = {BMC pulmonary medicine},
year = {2026},
month = jul,
doi = {10.1186/s12890-026-04510-w},
url = {https://europepmc.org/article/MED/42894161},
note = {CC BY-NC-ND},
}
RIS
TY - JOUR
TI - Pulmonary epithelioid hemangioendothelioma with pleural dissemination: a case report of prolonged diagnostic delay and the role of surgical biopsy.
AU - Rui Yan
AU - Weiwei Feng
AU - Lu Lu
AU - Yi Gong
AU - Guifang Wang
PY - 2026
DA - 2026/07/01
JO - BMC pulmonary medicine
DO - 10.1186/s12890-026-04510-w
UR - https://europepmc.org/article/MED/42894161
AB - Background Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumor with low incidence and nonspecific clinical and radiological features, frequently leading to misdiagnosis and delayed diagnosis.Case presentation We report a 32-year-old man with bilateral pulmonary nodules that were followed for 6 years before the development of a new left lower lobe mass, pleuritic chest pain, dyspnea, and massive pleural effusion in 2025. Initial imaging revealed diffuse miliary nodules that remained relatively indolent for several years. Two transbronchial lung biopsies (TBLB) and repeated pleural fluid cytology failed to establish a diagnosis. Definitive diagnosis was achieved only after video-assisted thoracoscopic surgery (VATS), with immunohistochemical support for endothelial differentiation and molecular confirmation of the WWTR1-CAMTA1 fusion gene. The patient underwent surgery, systemic therapy, and intrapleural treatment, followed by maintenance sirolimus. Disease remained clinically and radiologically stable during 6 months of follow-up.Conclusions PEH presents substantial diagnostic challenges because of its indolent course and nonspecific clinical and radiological features. For patients with unexplained multiple pulmonary nodules and pleural involvement, especially when minimally invasive investigations remain repeatedly nondiagnostic, early surgical biopsy combined with endothelial immunophenotyping, and molecular testing is essential for timely diagnosis and optimal management.
SN - 1471-2466
ER -