Treatment of hepatic epithelioid hemangioendothelioma: a single-institution experience with 25 cases.
OBJECTIVE
To examine treatment of hepatic epithelioid hemangioendothelioma (EHE), a rare vascular tumor with a variable course. Treatment modalities at our institution include liver resection, transplantation, and catheter-based therapies.


METHODS
Retrospective review of 25 patients treated for hepatic EHE (1976-2007). We examined treatment modality, overall survival, complications, and clinicopathologic characteristics.


RESULTS
Of the 25 patients treated for hepatic EHE, 17 underwent liver transplantation (LT); 4, transcatheter arterial chemoembolization (TACE); 2, resection; and 2, TACE followed by LT. Twelve patients (48%) were male. The median age at diagnosis was 38 years (range, 9 months to 72 years). Mean overall survival was 167 (95% confidence interval [CI], 123-212) months, with 172 (124-220) months in the LT group and 83 (54-112) months in the TACE group. The 2 patients in the resection group remain alive after 19 and 71 months. The 2 patients treated with TACE followed by LT died after 13 and 113 months. Extrahepatic disease was identified as a predictor of outcome. Patients with extrahepatic disease treated with TACE fared better than those treated with surgical approaches (mean survival, 83.0 [95% CI, 54.2-111.8] vs 38.8 [23.7-53.8] months; P = .12).


CONCLUSIONS
Hepatic EHE is a rare tumor that can be treated with surgical or nonsurgical approaches. In our experience, LT is used for patients with advanced local disease, whereas TACE is the primary modality when extrahepatic disease or comorbid conditions prohibiting LT are present. To our knowledge, this is the largest single-institution experience describing the various therapeutic modalities in the treatment of hepatic EHE.
Cardinal
Jon
J
Thomas E. Starzl Transplantation Institute, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
de Vera
Michael E
ME
Marsh
James W
JW
Steel
Jennifer L
JL
Geller
David A
DA
Fontes
Paulo
P
Nalesnik
Michael
M
Gamblin
T Clark
TC
D000046:Academic Medical Centers; D000293:Adolescent; D000328:Adult; D000368:Aged; D001707:Biopsy, Needle; D002423:Cause of Death; D016461:Chemoembolization, Therapeutic / Q000379:methods; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006390:Hemangioendothelioma / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D006498:Hepatectomy / Q000379:methods; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D053208:Kaplan-Meier Estimate; D008113:Liver Neoplasms / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D016031:Liver Transplantation / Q000379:methods; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011183:Postoperative Complications / Q000401:mortality / Q000503:physiopathology; D016016:Proportional Hazards Models; D035583:Rare Diseases; D012189:Retrospective Studies; D018570:Risk Assessment; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed; D055815:Young Adult