Multiple Hepatic Epithelioid Hemangioendothelioma; A Rare Case Report

Introduction: Epithelioid hemangioendothelioma (EHE) is a very rare vascular tumor. It is characteristically locally aggressive and can arise from soft tissue and bone. There is diagnostic confusion between EHE and other benign and malignant vascular tumors. A WWTR1-CAMTA1 translocation fusion gene is found in 90% of conventional EHE.  Case Report : A 44-year-old woman was found to have two hepatic lesions after evaluation for right-sided hypochondrial pain. Biopsy of the largest lesion was found to be EHE. Immunohistochemistry showed positivity of tumor cells for CD31 & CD34. A staging CT scan and PET scan were performed and both ruled out other lesions elsewhere in the body. A right hepatectomy was performed. Three lesions were found. All were EHE. The first nodule was found near the portal vein and measured 3x2.6x2cm. The second and third nodules were 1.5x1.5cm and 8mm respectively. After surgery, the patient is under regular follow-up and monitoring. She is still free from any recurrence.  Discussion : Multiple hepatic epithelioid hemangioendothelioma (EHE) is a diagnostic challenge when there is still a lack of appropriate active and reproducible treatment options for it.  Studies show that most EHE present with multiple liver masses. The definitive diagnosis of EHE is based on histopathology ranging from paucicellular to moderately cellular lesions. On FDG-PET scans, EHE typically show moderate FDG activity. The diagnosis of our case was challenging due to the presence of an occult mass that was not detectable on any imaging modality. Both liver transplantation and liver resection have been discussed; the latter strategy was used in our case. Conclusion: EHE is a very rare vascular tumor that arises from the cells lining the blood vessels. EHE is a locally aggressive tumor. Hepatic EHE usually presents as multiple masses in the liver. It has its own immunohistochemical and genetic characteristics. Surgery is the mainstay of treatment.