간이식 시대의 소아 간모세포종의 치료 성적
Impact of liver transplantation on survival outcome of hepatoblastoma
Abstract
Background: Effective chemotherapy and progress in surgical techniques have been improving the outcome of the patients with hepatoblastoma. Especially, liver transplantation (LT) permits long-term survival of the patients with unresectable tumor, whose outcome is extremely dismal without LT. This study was to analyze the outcomes of hepatoblastoma, highlighting on the impact of liver transplantation. Methods: We retrospectively analyzed the medical records of 64 children with hepatoblastoma treated at Asan Medical Center between 1991 and 2014. Liver transplantation program for hepatoblastoma started at Asan Medical Center in 2007. Twenty-nine and 35 patients were treated before and after 2007, respectively. Results: According to the PRETEXT staging system, 3 patients were PRETEXT I, 31 were II, 13 were III, and 17 were IV. Twenty-five patients had distant metastases at diagnosis. Of 64 patients, 5 underwent primary tumor resection, and 59 received preoperative chemotherapy. Of those 59 patients, 12 patients died of progressive disease before completion of treatment. Of 37 patients who completed treatment (including 12 patients who received LT), 6 patients recurred, and 4 of them died of disease progression. Two patients died of treatment-related myelodysplastic syndrome and pre-existing congenital adrenal hyperplasia. The 5-year overall survival (OS) and event-free survival (EFS) rates were 66.8% and 64.7%, respectively. PRETEX stage and metastasis at diagnosis were significantly associated with OS ( PRETEXT I vs. II vs. III vs. IV: 100% vs. 79.7% vs. 55.9% vs. 43.9%, P = 0.004; no metastasis vs. metastasis: 79.6% vs. 47.1%, P = 0.026). The 5-year OS after 2007 was significantly better than that before 2007 (80.7% vs. 55.2%, P = 0.045). The prognostic impact of PRETEXT stage was observed only before 2007, and not after 2007( PRETEXT I vs. II vs. III vs. IV: 100% vs. 73.3% vs. 50.0% vs. 14.3% before 2007, P = 0.008; 100% vs. 93.8% vs. 60.0% vs. 67.5% after 2007, P = 0.379). Furthermore, impact of metastasis at diagnosis on survival tended to be less prominent after 2007 (no metastasis vs. metastasis: 71.4% vs. 40.0%, P = 0.147 before 2007; 83.6% vs. 70.0%, P = 0.405 after 2007). Conclusion: Our result demonstrated that LT could overcome previously known poor prognostic factors of hepatoblastoma such as PRETEXT stage and metastasis.