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
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