Abstract / Summary
Epithelioid hemangioendothelioma (EHE) is an exceptionally rare vascular neoplasm that often represents a diagnostic challenge.It predominantly affects women and most commonly involves the liver, lungs, and bones.To date, no specific predisposing factors have been clearly identified.Data regarding the management of metastatic EHE remain limited, and standardized therapeutic strategies are lacking.At early stages, surgical resection, including amputation when necessary, with or without radiotherapy, remains the mainstay of treatment to prevent disease dissemination.The medical oncologist plays a crucial role in this rare entity, especially in advanced stages, by guiding a multidisciplinary, individualized approach.We report the case of a 61-year-old patient who presented with painful swelling on the lateral aspect of the right knee.Histopathological examination of bone and soft tissue biopsies favored the diagnosis of a high-risk vascular tumor consistent with epithelioid hemangioendothelioma. Staging investigations revealed widespread metastatic disease involving the liver, bones, lungs, and intestinal tract.This case highlights the aggressive potential of EHE and underscores the need for early oncological involvement and the development of evidence-based therapeutic strategies.Despite its rarity, advancing clinical research and establishing treatment recommendations are essential to improve outcomes in patients with advanced EHE.