Hepatic Epithelioid Hemangioendothelioma: A Case Report of an Extremely Rare Tumor

Abstract

Epithelioid hemangioendothelioma is a very rare vascular tumour, with fewer than 500 cases described worldwide. Diagnosis is often multifocal or at an advanced stage with non-specific symptoms. Pathological examination by biopsy is crucial to confirm the diagnosis. The liver is the most frequent site of predilection, followed by the lungs and bones. The etiology of hepatic epithelioid hemangioendothelioma is poorly understood and is not linked to chronic liver disease, with a heterogeneous and unpredictable course depending on the individual. Given the rarity of this cancer, there are no established treatment standards for this type of tumor of intermediate malignancy. Surgical resection is performed in the case of unifocal disease, and orthotopic liver transplantation remains an option in the case of multifocal and/or unresectable disease, with high survival rates and disease free survival (DFS). Transcatheter arterial chemoembolization (TACE) and chemotherapy/radiotherapy regimens are other treatment possibilities. However, surveillance may be proposed for asymptomatic patients with a slow progression. We report the case of a patient followed for a multifocal EHE with pulmonary metastases, discovered following pain in the right hypochondrium. Morphological and immunohistochemical examination confirmed the diagnosis of hepatic epithelioid hemangioendothelioma. Given the moderate symptoms, surveillance was recommended for our patient with a good clinical course and radiological stability for 3 years.