A rare case report of epithelioid hemangioendothelioma involving both lungs and right atrium with multi-system involvement.

Epithelioid hemangioendothelioma (EHE) is a rare low-grade malignant vascular neoplasm characterized by local aggressiveness and metastatic potential. Clinically, EHE may involve multiple anatomical sites-including the lungs, liver, bones, and soft tissues-either synchronously or metachronously. Its non-specific clinical manifestations often complicate early diagnosis. Given the extreme rarity of documented cases in the literature, we report a rare case of right atrial EHE with concurrent bilateral pulmonary and extensive systemic metastases. Furthermore, we present a comprehensive literature review to explore the diagnostic and therapeutic challenges associated with this disease. We particularly emphasize the necessity of a thorough diagnostic evaluation encompassing both pathological and imaging modalities to ensure accurate characterization and diagnosis of the tumor in this case.