Liver Transplantation for Diffuse Hepatic Epithelioid Hemangioendothelioma

Abstract

Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare vascular tumor. His treatment is not clearly defined. In diffuse HEHE, liver transplantation (LT) is the only possible surgery. Male, 60 years old, medical history: allergic to acetylsalicylic acid and hiatal hernia, presents anorexia, asthenia, weight loss and abdominal distension. Ultrasound, abdominal CT, MRI and PET-CT showed multiple bilobar nodules that diffusely affect liver parenchyma, ascites without portal thrombosis and no extrahepatic disease. FNAC: HEHE. The patient presents clinical and liver function worsening due to thrombosis of the central and right portal branch. He was evaluated in the Transplant Committee and listed with MELD 27. In July 2021, he underwent LT with vena cava preservation. Cold ischemia time (275 minutes), warm ischemia (32 minutes) and total surgery (288 minutes). A portal thrombectomy and hilar lymphadenectomy was also performed. Immunosuppression is started with corticosteroids, mycophenolate, and basiliximab (single dose). On the third day started with Advagraf®. He was discharged on the 11th day post-LT without Clavien-Dindo≥ IIIa complications. Pathology: diffuse HEHE with intrahepatic venous invasion and gallbladder wall; portal tumor thrombus and metastases in 5 /23 lymph nodes excised. Positivity for CD31, CD34 and negative for CKAE1/AE3. Low proliferative activity with Ki67:3%. Diffuse HEHE is an extraordinary rare tumor. The survival of HEHE after LT, according to the ELTR, is 88.6% and 79.5% at 1-year and 5-years respectively. 25% of LT patients have recurrence (median:18 months). The results obtained with LT are excellent, although the risk of recurrence is not negligible.