Long-term results after liver transplantation for primary hepatic epithelioid hemangioendothelioma.
BACKGROUND
Hepatic epithelioid hemangioendothelioma (PHEHE) is a multifocal, low-grade malignant neoplasia characterized by its epithelial-like appearance and vascular endothelial histogenesis. The outcome of 16 patients treated with orthotopic liver transplantation (OLT) is the subject of this report.


METHODS
A retrospective study of 16 patients with HEHE (7 men, 9 women) with ages ranging from 24 to 58 years (mean 37 +/- 10.6 years). Follow-up intervals ranged from 1 to 15 years (median of 4.5 years).


RESULTS
Actual patient survival at 1, 3, and 5 years was 100, 87.5, and 71.3%, respectively. Disease-free survival at 1, 3, and 5 years was 81.3, 68.8, and 60.2%, respectively. The 90-day operative mortality was 0. Involvement of the hilar lymph nodes or vascular invasion did not affect survival. The 5-year survival of HEHE compares favorably with that of hepatocellular carcinoma at the same stage (stage 4A): 71.3 versus 9.8% (p = 0.001)


CONCLUSIONS
The long-term survival obtained in this series justifies OLT for these tumors even in the presence of limited extrahepatic disease.
Madariaga
J R
JR
Department of Surgery, University of Pittsburgh School of Medicine, Pennsylvania, USA.
Marino
I R
IR
Karavias
D D
DD
Nalesnik
M A
MA
Doyle
H R
HR
Iwatsuki
S
S
Fung
J J
JJ
Starzl
T E
TE
D000328:Adult; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000401:mortality / Q000601:surgery*; D006801:Humans; D008113:Liver Neoplasms / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D015996:Survival Rate; D013997:Time Factors