title: Pediatric liver transplantation for primary malignant liver tumors with a focus on hepatic epithelioid hemangioendothelioma: the UNOS experience.
abstract: Treatment for HEH does not follow a standardized algorithm. From clinical experience, it is assumed that pediatric patients with HEH will fare as well as other common pediatric liver tumors post-OLT. The UNOS dataset was examined for patients with pediatric OLT between 1987 and 2007. Patients were grouped into non-tumors, HB, HCC, HEH, and rare liver tumors. COD analysis was calculated using Fisher's exact test. Patient, allograft, and recurrence-free survival were compared using Kaplan-Meier curves and log-rank tests. A total of 366 patients with pediatric OLT were identified with primary liver tumors (HB - 237, HCC - 58, HEH - 35, other - 36). HEH patient survival (five yr: 60.6%) was poorer than non-tumor OLTpatient survival (five yr: 84.4%). Survival was worse when compared to HB (five yr: 72%) and rare liver tumors (five yr: 78.9%), but better than HCC (five yr: 53.5%). Allograft survival in HEH (five yr: 50.1%) lies between HB (five yr: 63.6%) and HCC (five yr: 42.8%). COD analysis demonstrates recurrence as a major cause in HB and HCC, but not for HEH or other liver tumors. The data suggest that patient survival may not be as high as previously believed and further investigation is warranted.
authors.affiliation: Michael E. DeBakey Department of Surgery, Division of Abdominal Transplantation and Hepatobiliary Surgery, Baylor College of Medicine, Houston, TX 77030, USA.
authors.forename: Jacfranz J
authors.initials: JJ
authors.lastname: Guiteau
authors.forename: Ronald T
authors.initials: RT
authors.lastname: Cotton
authors.forename: Saul J
authors.initials: SJ
authors.lastname: Karpen
authors.forename: Christine A
authors.initials: CA
authors.lastname: O'Mahony
authors.forename: John A
authors.initials: JA
authors.lastname: Goss
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D018323:Hemangioendothelioma, Epithelioid / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality; D008297:Male; D009364:Neoplasm Recurrence, Local; D012307:Risk Factors; D016019:Survival Analysis; D016896:Treatment Outcome