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

Abstract

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.

Hepatic epithelioid hemangioendothelioma successfully treated with living donor liver transplantation: A case report and literature review

“…We summarized 14 studies that reported on LT for HEH (Table ) . While there were cases where death occurred shortly after LT due to early recurrence, there were also cases of long‐term survival even if recurrence was observed after LT.…”

“…We summarized 14 studies that reported on LT for HEH ( Table 2). [8][9][10][11][12][22][23][24][25][26][27][28][29][30] While there were cases where death occurred shortly after LT due to early recurrence, there were also cases of long-term survival even if recurrence was observed after LT. In the cases with long-term survival, the time to recurrence was longer; thus, the tumor's malignant potential may have been lower.…”