간이식 시대의 소아 간모세포종의 치료 성적

간이식 시대의 소아 간모세포종의 치료 성적

Impact of liver transplantation on survival outcome of hepatoblastoma

(구연):
Release Date : 2014. 10. 25(토)
Kyung-Nam Koh1, Jin Kyung Suh1, Seong Wook Lee1, Darae Lee1, Seak Hee Oh2, Kyung Mo Kim2, Jung-Man Namgoong3, Dae-Yeon Kim 3, Young Ah Cho4, Jin Sung Lee4, Se-Hoon Choi5, Dong Kwan Kim5, Ho Joon Im1 , Jong Jin Seo1
Asan Medical Center Children's Hospital, University of Ulsan College of Medicine Division of Pediatric Hematology/Oncology, Department of Pediatrics1
Asan Medical Center Children's Hospital, University of Ulsan College of Medicine Department of Pediatric Surgery2
Asan Medical Center Children's Hospital, University of Ulsan College of Medicine Division of Pediatric Gastroenterology and Nutrition, Department of Pediatrics3
Asan Medical Center, University of Ulsan College of Medicine Department of Radiology4
Asan Medical Center, University of Ulsan College of Medicine Department of Thoracic and Cardiovascular Surgery5
고경남1, 서진경1, 이성욱1, 이다래1, 오석희2, 김경모2, 남궁정만3, 김대연3, 조영아4, 이진성4, 최세훈5, 김동관5, 임호준1 , 서종진 1
울산의대 서울아산병원 어린이병원 소아종양혈액과1
울산의대 서울아산병원 어린이병원 소아외과2
울산의대 서울아산병원 어린이병원 소아소화기영양과3
울산의대 서울아산병원 영상의학과4
울산의대 서울아산병원 흉부외과5

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.

Keywords: hepatoblastoma, liver transplantation,