Aggressive Recurrence of Primary Hepatic Epithelioid Haemangioendothelioma after Liver Transplantation

Abstract

HEHE is a rare neoplasm of vascular origin that occurs in the liver; UNOS reported a favorable outcome after liver transplantation in 110 patients with 1-year and 5-year survival of 80% and 64%. Case Report. A 40-year-old lady presented with a three-month history of right upper abdominal pain with nausea, vomiting, and significant loss of weight associated with scleral icterus and progressive abdominal distension. Examination revealed jaundice, hepatomegaly, and ascites. Serum bilirubin was 26.5 mg/dL and ALP was 552 CT. Abdomen and pelvis showed diffuse infiltrative neoplastic process of the liver with a mass effect and stretching of the hepatic and portal veins, in addition to bile duct dilatation. Viral hepatitis markers were negative and serum alpha fetoprotein was within reference range. Liver biopsy was consistent with HEHE, with positive endothelial markers (CD31, CD34, and factor VIII-related antigen). She underwent living related liver transplantation on June 2013 and was discharged after 20 days with normal liver enzymes. Four months later, she presented with diffuse disease recurrence. Liver biopsy confirmed disease recurrence; she received supportive treatment and unfortunately she died 2 weeks later. Conclusion. HEHE can have rapid and aggressive recurrence after liver transplantation.

Liver Transplantation in Malignancies: A Comprehensive and Systematic Review on Oncological Outcome

“…However, HEHE belongs to uncommon liver lesions with not always typical imaging features, and a biopsy for its diagnosis is therefore necessary. Outcome after LT in HEHE must be assessed further, as recurrence is an unpredictable event after LT [11] (Table 3).…”

“…HCC is the ninth most common malignancy among women worldwide and is the fifth most common malignancy in men [72]. HCC within the setting of cirrhosis is given a higher priority, and thus, it is a rapidly growing indication for LT [11]. Both the detection and treatment of HCC have evolved significantly during the last decade and comprise local ablation, chemoembolization, and laparoscopic liver resection toward transplantation [73] and promising trials on adjuvant chemotherapy [74] after LT for a curative intention.…”

The challenging surgical management of hepatic epithelioid hemangioendothelioma: a narrative review

“…Several surgical approaches, like hepatic artery ligation, hepatic resection, or LT, have been utilized to cure HEHE (Table 2) (14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)30,32,33,50,63). Hepatectomy is adopted to cure localized single lesions, whilst LT represents the treatment of choice to treat multifocal diseases (51).…”