Sakil Kulkarni1, David G Brauer2, Yumirle Turmelle3, Janis Stoll3, Michelle Nadler3, William C Chapman2, Maria B Doyle2, Adeel S Khan2. 1. Department of Pediatrics, Division of Pediatric Hepatology, St. Louis Children's Hospital, 660 S. Euclid Avenue, Campus Box 8112, St. Louis, MO, 63110, USA. Sakil.Kulkarni@wustl.edu. 2. Department of Surgery, Division of Abdominal Transplant Surgery, Washington University in St Louis, St. Louis, MO, USA. 3. Department of Pediatrics, Division of Pediatric Hepatology, St. Louis Children's Hospital, 660 S. Euclid Avenue, Campus Box 8112, St. Louis, MO, 63110, USA.
Abstract
INTRODUCTION: Hepatoblastoma (HB) is a rare childhood malignancy with hepatic resection (HR) or liver transplantation (LT) providing the best chance of cure. In this study, we analyze the National Cancer Database lacks (NCDB) to compare outcomes following HR and LT for HB. METHODS: Review of the US experience with surgical (HR and LT) management of pediatric (< 18 years) HB over the last decade (2004-2014) using data extracted from the NCDB. RESULTS: A total of 628 children underwent surgical treatment for HB during the study period: HR in 525 (83.6%) and LT in 103 (16.4%). The two groups were comparable for age, sex, race, tumor size, and metastatic disease at initial diagnosis. LT group had significantly higher number of patients with bilobar disease (40 vs 21%; p < 0.001), longer median time from diagnosis to surgery (120 vs 78 days; p < 0.001), and longer post-operative length of stay (LOS) (14 vs 6 days; p < 0.001). There were no differences in rates of 30-day readmission and 30- or 90-day mortality between groups. Both groups had comparable 5-year overall survival (OS) (84.1% HR vs 80.0% LT; p = 0.4). Univariate analysis identified metastatic disease at initial presentation (HR 2.56, CI 1.51-4.35) and age ≥ 4 years (HR 2.68, CI 1.5-4.7) as risk factors for worse overall 5-yr OS, while administration of adjuvant chemotherapy was associated with improved 5-yr OS (92.3% with chemo vs 85.4% without chemo; HR 0.51, CI 0.31-0.84). CONCLUSION: The outcome of HB has improved compared with historical controls. Age at presentation, metastatic disease, and post-operative chemotherapy impact outcomes.
INTRODUCTION:Hepatoblastoma (HB) is a rare childhood malignancy with hepatic resection (HR) or liver transplantation (LT) providing the best chance of cure. In this study, we analyze the National Cancer Database lacks (NCDB) to compare outcomes following HR and LT for HB. METHODS: Review of the US experience with surgical (HR and LT) management of pediatric (< 18 years) HB over the last decade (2004-2014) using data extracted from the NCDB. RESULTS: A total of 628 children underwent surgical treatment for HB during the study period: HR in 525 (83.6%) and LT in 103 (16.4%). The two groups were comparable for age, sex, race, tumor size, and metastatic disease at initial diagnosis. LT group had significantly higher number of patients with bilobar disease (40 vs 21%; p < 0.001), longer median time from diagnosis to surgery (120 vs 78 days; p < 0.001), and longer post-operative length of stay (LOS) (14 vs 6 days; p < 0.001). There were no differences in rates of 30-day readmission and 30- or 90-day mortality between groups. Both groups had comparable 5-year overall survival (OS) (84.1% HR vs 80.0% LT; p = 0.4). Univariate analysis identified metastatic disease at initial presentation (HR 2.56, CI 1.51-4.35) and age ≥ 4 years (HR 2.68, CI 1.5-4.7) as risk factors for worse overall 5-yr OS, while administration of adjuvant chemotherapy was associated with improved 5-yr OS (92.3% with chemo vs 85.4% without chemo; HR 0.51, CI 0.31-0.84). CONCLUSION: The outcome of HB has improved compared with historical controls. Age at presentation, metastatic disease, and post-operative chemotherapy impact outcomes.
Entities:
Keywords:
Hepatoblastoma; Liver transplantation; National Cancer Database; Outcome; Resection
Authors: M Wilschanski; P Chait; J A Wade; L Davis; M Corey; P St Louis; A M Griffiths; L M Blendis; S P Moroz; L Scully Journal: Hepatology Date: 1995-11 Impact factor: 17.425