Cor Vasa 2026, 68(4):509-512 | DOI: 10.33678/cor.2026.076

Diseminovaný epiteloidní hemangioendoteliom jako vzácná příčina nádorového postižení srdce.

Radka Světlíková^a, Jan Opatrný^a, Jan Baxa^b, Richard Rokyta^a

^a Kardiologická klinika, Lékařská fakulta v Plzni, Univerzita Karlova a Fakultní nemocnice Plzeň, Plzeň, Česká republika

^b Nemocnice Hořovice, AKESO holding a.s., Hořovice, Česká republika

Epithelioid hemangioendothelioma (EHE) is a rare mesenchymal tumor of vascular origin with a heterogeneous clinical course. It most commonly affects the liver, lungs, bones, and soft tissues, whereas cardiac involvement is an exceptionally rare manifestation of the disease and may mimic benign primary cardiac tumors. We present the case of a 29-year-old man with no significant personal or family medical history who was admitted for progressively worsening dyspnea. Initial examination revealed bilateral pleural effusion, pericardial effusion, and an intracavitary mass in the left atrium measuring up to 40 mm, which was considered suspicious for a myxoma based on its echocardiographic morphology. Further imaging revealed a tumor mass in the posterior mediastinum infiltrating the left atrium, pleural involvement, and multiple lesions in the lung parenchyma and skeleton. A hematologic malignancy was considered in the differential diagnosis but was not confirmed. The definitive diagnosis of disseminated epithelioid hemangioendothelioma was established by histopathological and immunohistochemical examination of a bone lesion demonstrating CAMTA1 positivity.1. Despite the initiation of palliative systemic therapy, the disease progressed rapidly, and the patient died four months after the diagnosis was established. This case highlights the need to consider a malignant etiology even in apparently typical intracardiac masses, particularly in the presence of systemic manifestations. A multimodality imaging approach combined with histopathological verification is essential for establishing the correct diagnosis.1,2 © 2026, ČKS.

Keywords: Echocardiography, Epithelioid hemangioendothelioma, Heart neoplasms, Mediastinal neoplasms, Positron-emission tomography

Received: August 6, 2026; Accepted: August 18, 2026; Prepublished online: August 19, 2026; Published: August 31, 2026  Show citation