Hepatic epithelioid hemangioendothelioma: case series of a rare vascular tumor mimicking metastases

Abstract

Background Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature. Methods All cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained. Results Seven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon. Conclusion Clinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.

Hepatic Epithelioid Hemangioendothelioma Discovered Incidentally on Computerized Tomography

“…Histologically, HEHE manifests as nests and cords of spindle to epithelioid cells with mild to moderate atypia, abundant pale to eosinophilic cytoplasm, indistinct cell boundaries, and intracytoplasmic vacuolations in a fibromyxoid stroma. 4 Older lesions appear sclerotic, calcified, or necrosed. 2 …”

“…There is no consensus for a gold standard of treatment, however, there is agreement that treatment should consider the involvement of both liver lobes, lesion size/number, and disease progression. 4 Treatment includes radiation, chemotherapy, liver resection, and liver transplant. While agents targeting anti-angiogenesis have had the most success in clinical trials, common chemotherapeutics include doxorubicin, vincristine, interferon-alpha, 5-fluorouracil, thalidomide, and monoclonal antibodies against vascular endothelial growth factor.…”

“…Liver transplant is the treatment of choice for diffuse, multifocal lesions, and rapidly progressive disease. 4 , 6 …”

“…While 25 – 40% of patients are asymptomatic, common presenting symptoms include right upper-quadrant pain, fatigue, nausea, vomiting, weakness, ascites, anorexia, and weight loss. 2 – 4 Up to 37% of patients have distant metastases at the time of diagnosis and rapid progression is seen in up to 75% of cases where there are multifocal lesions. 2 , 4 This report presents a unique case of HEHE found incidentally on computerized tomography (CT).…”

“… 2 – 4 Up to 37% of patients have distant metastases at the time of diagnosis and rapid progression is seen in up to 75% of cases where there are multifocal lesions. 2 , 4 This report presents a unique case of HEHE found incidentally on computerized tomography (CT).…”