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.
 Richardson WS, Stefanidis D, Chang L, Earle DB and Fanelli
RD. The role of diagnostic laparoscopy for chronic abdominal
conditions: an evidencebased review. SurgEndosc 2009; 23:
 Mehrabi A, Kashfi A, Fonouni H, et al. Primary malignant
hepatic epithelioidhemangioendothelioma: a comprehensive
review of the literature with emphasis on the surgical therapy.
Cancer 2009;107: 21082121, 2006
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