Clinical experience with primary hepatic epithelioid hemangioendothelioma: retrospective study of 33 patients.
BACKGROUND
This multicenter-based retrospective study aimed to investigate the prognostic factors and report our experiences with the diagnosis and treatment of hepatic epithelioid hemangioendothelioma (HEHE), a rare malignant vascular tumor.


METHODS
A total of 33 patients with HEHE from two centers between 2004 and 2011 were retrospectively reviewed with respect to their clinical, radiologic, and pathologic characteristics; treatment modalities and outcomes; and potential prognostic factors.


RESULTS
A total of 17 patients underwent liver resections (LRs) alone, 12 patients had transcatheter arterial chemoembolization (TACE) alone, three patients had LR followed by TACE, and one patient underwent liver transplantation (LT). The difference of overall survival (OS) between LR and TACE was not significant (p = 0.499). Older patients [≥47 years, n = 17; p = 0.035, hazard ratio (HR) = 7.0), those with symptoms (n = 17; p = 0.001, HR = 86.5], and those with an elevated serum CA19-9 level (>37 U/ml, n = 5; p = 0.018, HR = 5.0) had a poorer OS, according to univariate analysis. The presence of symptoms was validated as a prognostic factor (p = 0.012) by multivariate analysis.


CONCLUSIONS
Liver resection and TACE have comparable outcomes in HEHE patients. The presence of symptoms indicates a poor prognosis. Older age and elevated serum CA19-9 are potential negative impact factors on outcome.
Liver Cancer Institute and Zhongshan Hospital, Fudan University, 136 Yi Xue Yuan Road, Shanghai 200032, People's Republic of China. w.lr@hotmail.com
Long-Rong
LR
Wang
Jia-Min
JM
Zhou
Yi-Ming
YM
Zhao
Hong-Wei
HW
He
Zong-Tao
ZT
Chai
Miao
M
Wang
Yuan
Y
Ji
Yi
Y
Chen
Chen
C
Liu
Hui-Chuan
HC
Sun
Wei-Zhong
WZ
Wu
Qing-Hai
QH
Ye
Jian
J
Zhou
Jia
J
Fan
Zhao-You
ZY
Tang
Lu
L
Wang
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000628:therapy; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D055815:Young Adult