Hepatic epithelioid haemangioendothelioma: is transplantation the only treatment option?
BACKGROUND
Hepatic epithelioid haemangioendothelioma (HEH) is a rare vascular neoplasm with unpredictable clinical behaviour.


OBJECTIVE
To compare overall survival (OS) and disease-free survival (DFS) between liver resection (LR) and orthotopic liver transplantation (OLT) for the treatment of HEH.


METHODS
Retrospective review of 30 patients with HEH treated at Mayo Clinic during 1984 and 2007.


RESULTS
Median age was 46 years with a female predominance of 2:1. Treatment included LR (n= 11), OLT (n= 11), chemotherapy (n= 5) and no treatment (n= 3). LR was associated with a 1-, 3- and 5-year OS of 100%, 86% and 86% and a DFS of 78%, 62% and 62%, respectively. OLT was associated with a 1-, 3- and 5-year OS of 91%, 73% and 73% and a DFS 64%, 46% and 46%, respectively. Metastases were present in 37% of patients but did not significantly affect OS. Important predictors of a favourable OS and DFS were largest tumour ≤ 10 cm and multifocal disease with ≤ 10 nodules.


CONCLUSIONS
LR and OLT achieve comparable results in the treatment of HEH. LR is appropriate for patients with resectable disease and favourable prognostic factors. OLT is appropriate for patients with unresectable disease and possibly those with unfavourable prognostic factors. Metastases may not be a contraindication to surgical treatment.
Grotz
Travis E
TE
Division of Gastroenterologic and General Surgery, Mayo Clinic, Rochester, MN 55905, USA.
Nagorney
David
D
Donohue
John
J
Que
Florencia
F
Kendrick
Michael
M
Farnell
Michael
M
Harmsen
Scott
S
Mulligan
David
D
Nguyen
Justin
J
Rosen
Charles
C
Reid-Lombardo
Kaye M
KM
D000328:Adult; D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D016009:Chi-Square Distribution; D018572:Disease-Free Survival; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006498:Hepatectomy / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D008113:Liver Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D010166:Palliative Care; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D014481:United States; D055815:Young Adult
D000970:Antineoplastic Agents