[Hepatic Epitheloid Hemangioendothelioma with Pulmonary Metastasis at Onset and Slow Progression. A Case Report with...

Epithelioid hemangioendothelioma (EHE) is an extremely rare vascular sarcoma, with an estimated incidence of 0.32 to 0.38 cases per million person-years. We present the case of an 18-year-old woman with slowly progressive metastatic EHE. The patient experienced persistent abdominal pain and sought emergency care on multiple occasions without receiving a final diagnosis. Three years later, imaging studies revealed multiple hepatic lesions, and a biopsy confirmed the diagnosis of EHE. Although therapeutic options such as surgical resection and chemotherapy were considered, an active surveillance approach was chosen due to the slow disease progression and the absence of severe symptoms. After 26 months of follow-up, the patient shows no signs of tumor progression and leads a normal life, with occasional abdominal pain responsive to symptomatic treatment. This case suggests that active surveillance may be a valid strategy for managing EHE in selected patients, allowing for the avoidance of overtreatment and the complications associated with aggressive medical-surgical interventions.