Hepatic epithelioid hemangioendothelioma: case report with resection and adjuvant bevacizumab-capecitabine

Abstract Epithelioid hemangioendothelioma of the liver is a rare vascular hepatic malignancy that often poses a diagnostic challenge. Its morphology appears intermediate between hemangioma and angiosarcoma. The clinical course may be aggressive, although with considerable variability. Surgical excision represents the mainstay of therapy when feasible. Given its rarity, no standard systemic therapy exists for unresectable or metastatic disease, and the role of adjuvant treatment remains undefined. We report the case of a 49-year-old woman with multifocal hepatic epithelioid hemangioendothelioma who underwent extensive surgical resection followed by adjuvant capecitabine and bevacizumab after detection of a micrometastasis in a cystic duct lymph node (pN1). The patient remains disease-free at a follow-up of at least 4 years.