Primary hemangioendothelioma of liver; report of a case and review of literature

ABSTRACT

Hepatic epithelioid hemangioendothelioma [HEH] is an exceedingly rare tumor of vascular origin with unknown etiology and unpredictable natural course and malignant potential. The disease presents with myriad of vague symptoms and can be diagnosed by ultrasound, CT, and MRI. Target shaped hepatic lesions due to hyperechoic center and hypoechoic peripheral rim are the diagnostic ultrasonic features. Hepatic capsular retractions due to extensive fibrosis can be identified by CT and MRI. The lesions are usually hypointense on T1-weighted images and hyperintense on T2-weighted images. There is no consensus on treatment of hepatic epithelioid hemangioendothelioma, which ranges from hepatic resection, hepatectomy, liver transplantation, chemo- and radiotherapy, and thermoablation. This case report presents a 36-year-old female, incidentally diagnosed to have hepatic epithelioid hemangioendothelioma, which was treated by right hepatectomy and was attended by favorable outcome: