Our experience of liver Epithelioid Hemangioendothelioma: from a misdiagnosis to liver transplantation with long term follow-up

Antonio Giuliani 1 , Bruno Amato 2 , Giuseppina Marino Marsilia 3 , Domenico Tafuri 4 , Antonio Ceriello 1 , Walter Santaniello 1 , Loredana Sodano 1 , and Aldo Rocca 1
  • 1 Unit of Hepatobiliary Surgery and Liver Transplant Center, Department of Gastroenterology and Transplantation,”A. Cardarelli” Hospital, Naples, Italy
  • 2 Department of Clinical Medicine and Surgery, University of Naples Federico II, Italy
  • 3 Department of Pathology, A. Cardarelli Hospital, Naples, Italy
  • 4 Department of Sport Sciences and Wellness, University of Naples “Parthenope”, Naples, Italy


Malignant Hepatic Epithelioid hemangioendothelioma (HEHE) is an uncommon vascular tumor of intermediate malignant potential. HEHE is a rare tumor and it is difficult to diagnose for surgeons, hepatologists, radiologists and pathologists. So, misdiagnosis with a delay of the treatment is not uncommon. We describe a case of a young woman with a diagnosis of HEHE made 6 years after the first evidence of liver mass with a very long term follow-up after surgical treatment. She had two diagnoses of Hepatocellurar carcinoma (HCC) and a diagnosis of Cholangiocarcinoma after three different fine needle biopsies. After clinical observation, a new laparoscopic core biopsy was performed. In a first time approach, considering clinical and radiological patterns, a diagnosis of Budd-Chiari Syndrome was finally made. For that the patient underwent an orthotopicliver transplantation (OLTx). The surgical sample histological analysis allowed a definitive diagnosis of HEHE. At last, at follow up 7 years after three OLTx the patient is still alive and in good health with no evidence of recurrence.

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