Epithelioid hemangioendothelioma with marked liver deformity and secondary Budd–Chiari syndrome: Pathological and radiological correlation

Abstract

A case of malignant epithelioid hemangioendothelioma of the liver in a 48-year-old woman with severe portal hypertension and marked deformity of the liver is presented. This woman had a history of mild liver dysfunction since the age of 30 years, and abdominal distention, esophageal varices, splenomegaly and ascites since October 1996. Imaging examinations revealed liver deformity with severe atrophy of the left lobe and the anterior segment of the right lobe. Celiac arteriography showed narrowing and upward deviation of the proper hepatic artery, and occlusion of the left and right anterior hepatic arteries. Since March 1997, hepatic venography showed stenosis in the right hepatic vein truncus. Budd-Chiari syndrome was clinically diagnosed. She died in June 1997. The autopsy disclosed massive tumor embolism in the left and right anterior portal branches, few in the hepatic artery, and occlusion of the left and right anterior hepatic arteries. The extensive tumor embolism resulted in portal hypertension, and atrophy of the left lobe. The anterior segment of the right lobe was probably caused by the occlusion of both the hepatic arteries and the portal veins. The posterior segment of the right lobe, without massive tumor embolism in its portal branch, appeared hypertrophic.

Surgical Treatment of Primary Hepatic Epithelioid Hemangioendothelioma

“…Many lesions are peripheral in location and extend to the liver capsule. Flattening or capsular retraction of the liver capsule due to fibrosis and compensatory hypertrophy of the unaffected liver segments may be diagnostic clues (10,11). On ultrasonography, the echogenicity of individual lesions is variable.…”

Hepatic epithelioid haemangioendothelioma: is transplantation the only treatment option?

“…Aetiologic risk factors are unknown, although HEH has occurred in association with oral contraceptive pills (OCPs), exposure to vinyl chloride or major hepatic trauma 3–7 . Clinical presentation is variable and non‐specific and ranges from an indolent course 6,7 to a rapidly progressive disease 8–12 . In nearly 25% of patients, HEH is diagnosed incidentally.…”

“…[3][4][5][6][7] Clinical presentation is variable and non-specific and ranges from an indolent course 6,7 to a rapidly progressive disease. [8][9][10][11][12] In nearly 25% of patients, HEH is diagnosed incidentally. The diagnosis of HEH is confirmed by immunostaining for factor VIII-related antigens and other endothelial markers on biopsy.…”

Angiomyolipoma of the Liver Causing Budd–Chiari Syndrome

“…A benign tumor causing Budd–Chiari syndrome is extremely rare. There are isolated reports of epithelioid hemangioendotheliomas causing secondary Budd–Chiari syndrome as a result of marked anatomical distortion of the liver 4 . To our knowledge, this is the first report of angiomyolipoma of the liver presenting as Budd–Chiari syndrome.…”