A RARE MALIGNANT NEOPLASM OF THE LUNG: EPITHELIOID HEMANGIOENDOTHELIOMA

Epithelioid hemangioendothelioma is a rare vascular tumor of endothelial origin. Pulmonary involvement is uncommon and diagnostically challenging. Due to its nonspecific clinical and radiological features, it may closely mimic primary or metastatic lung malignancies, frequently resulting in delayed diagnosis.We report a 57-year-old female who presented with a cough and was found to have a paramediastinal lung mass accompanied by bilateral multiple nodular lesions, pleural effusion, and mediastinal lymphadenopathy. Repeated bronchoscopic, transthoracic, and endobronchial ultrasound-guided biopsies were not diagnostic. Definitive diagnosis was achieved only after video-assisted thoracoscopic surgery, with histopathological and immunohistochemical findings confirming epithelioid hemangioendothelioma. The tumor demonstrated aggressive features, including multifocal parenchymal involvement, pleural dissemination, and a high mitotic index. The patient developed progressive respiratory failure and died in the early postoperative period despite intensive supportive management.This case emphasizes that pulmonary epithelioid hemangioendothelioma, although generally regarded as an indolent neoplasm, can exhibit rapidly progressive and fatal behavior in selected patients. Awareness of its aggressive potential and the role of surgical biopsy in establishing an accurate diagnosis is essential for appropriate clinical management.

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Journal
Journal of Istanbul Faculty of Medicine / İstanbul Tıp Fakültesi Dergisi
Published
2026-10-27
DOI
https://doi.org/10.26650/iuitfd.1854595
Primary Topic
Vascular Tumors and Angiosarcomas
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article
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article

A RARE MALIGNANT NEOPLASM OF THE LUNG: EPITHELIOID HEMANGIOENDOTHELIOMA

Mustafa Kuzucuoğlu, Servet Seçkin Tunç, Ebru Çakır, Serdar Şirzai et al.
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Journal of Istanbul Faculty of Medicine / İstanbul Tıp Fakültesi Dergisi
Vascular Tumors and Angiosarcomas
article

A RARE MALIGNANT NEOPLASM OF THE LUNG: EPITHELIOID HEMANGIOENDOTHELIOMA

Mustafa Kuzucuoğlu, Servet Seçkin Tunç, Ebru Çakır, Serdar Şirzai, Nİhat Berk Sarmış, Keramettin İbrahim Taylan, Mehmet Bekir Unal
article en

Abstract

Epithelioid hemangioendothelioma is a rare vascular tumor of endothelial origin. Pulmonary involvement is uncommon and diagnostically challenging. Due to its nonspecific clinical and radiological features, it may closely mimic primary or metastatic lung malignancies, frequently resulting in delayed diagnosis.We report a 57-year-old female who presented with a cough and was found to have a paramediastinal lung mass accompanied by bilateral multiple nodular lesions, pleural effusion, and mediastinal lymphadenopathy. Repeated bronchoscopic, transthoracic, and endobronchial ultrasound-guided biopsies were not diagnostic. Definitive diagnosis was achieved only after video-assisted thoracoscopic surgery, with histopathological and immunohistochemical findings confirming epithelioid hemangioendothelioma. The tumor demonstrated aggressive features, including multifocal parenchymal involvement, pleural dissemination, and a high mitotic index. The patient developed progressive respiratory failure and died in the early postoperative period despite intensive supportive management.This case emphasizes that pulmonary epithelioid hemangioendothelioma, although generally regarded as an indolent neoplasm, can exhibit rapidly progressive and fatal behavior in selected patients. Awareness of its aggressive potential and the role of surgical biopsy in establishing an accurate diagnosis is essential for appropriate clinical management.

Journal of Istanbul Faculty of Medicine / İstanbul Tıp Fakültesi DergisiVol. 89(4)
Izmir Kâtip Çelebi University (TR), Izmir Atatürk Eğitim ve Araştırma Hastanesi (TR)
Good health and well-being
Openalex Percentile: Top 16%
Vascular Tumors and Angiosarcomas
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A RARE MALIGNANT NEOPLASM OF THE LUNG: EPITHELIOID HEMANGIOENDOTHELIOMA — Mustafa Kuzucuoğlu, Servet Seçkin Tunç, et al. · Journal of Istanbul Faculty of Medicine / İstanbul Tıp Fakültesi Dergisi (2026) | TGRS Research Map | TGRS