Multifocal Pulmonary Epithelioid Hemangioendothelioma: A Metastatic Disease Mimicker
DOI:
https://doi.org/10.48729/pjctvs.588Keywords:
Hemangioendothelioma, Epithelioid, Lung, Thoracic Surgery, Pulmonary Medicine, PathologyAbstract
We present the case of a 79-year-old woman with multiple bilateral pulmonary nodules identified during an investigation for abdominal pain, initially suspected to represent metastatic disease. Extensive clinical, laboratory, and imaging workup failed to reveal a primary origin. After two non-diagnostic transthoracic biopsies, the patient underwent single-port video-assisted thoracoscopic surgery (VATS) with excision of two nodules from the upper and lower lobes of the right lung. Histopathological examination revealed an epithelioid hemangioendothelioma (EHE), a rare vascular neoplasm. This case illustrates the diagnostic process and pathological features of pulmonary EHE, with a brief clinical overview.
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Copyright (c) 2026 Portuguese Journal of Cardiac Thoracic and Vascular Surgery

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