Institute of Liver Disease and Transplantation, Sree Balaji Medical College Hospital, Bharat Institute of Higher Education and Research, Chennai, India.
Ray of Light Foundation, Kanchi Kamakoti Children's Health Institute Laboratory and Diagnostic Services (CHILDS) Trust Hospital, CHILDS Trust Medical Research Foundation, Chennai, India.
BJS Open. 2020 Aug;4(4):630-636. doi: 10.1002/bjs5.50297. Epub 2020 May 7.
Outcomes of high-risk hepatoblastoma have been dismal, especially in resource-challenged countries where access to chemotherapy and paediatric liver transplantation is limited for the underprivileged. This study aimed to assess the results of treatment of high-risk hepatoblastoma in a tertiary centre, including patients who had non-transplant surgical procedures in the form of extended resection.
A review of patients with high-risk hepatoblastoma treated between January 2012 and May 2018 was carried out. Perioperative data and long-term outcomes were analysed.
Of 52 children with hepatoblastoma, 22 were considered to have high-risk hepatoblastoma (8 girls and 14 boys). The mean(s.d.) age at diagnosis was 35(20) months. Of these 22 children, five died without surgery. Of the remaining 17 who underwent surgery, six had a resection (4 right and 2 left trisectionectomies) and 11 underwent living-donor liver transplantation. Median follow-up was 48 (range 12-90) months. Thirteen of the 17 children were alive at last follow-up and four developed disseminated disease (3 had undergone liver transplantation and 1 liver resection). The overall survival rate at 1, 3 and 5 years was 77, 64 and 62 per cent for the whole cohort with high-risk hepatoblastoma. In children who had surgery, 1-, 3- and 5-year survival rates were 91, 82 and 73 per cent for transplantation and 100, 83 and 83 per cent for resection. There was no difference in survival between the two surgical groups.
Excellent results in the treatment of high-risk hepatoblastoma are possible, even in resource-challenged countries.
高危型肝母细胞瘤的治疗效果一直较差,特别是在资源有限的国家,贫困患儿获得化疗和小儿肝移植的机会有限。本研究旨在评估三级中心治疗高危型肝母细胞瘤的结果,包括接受扩大切除术的非移植手术治疗的患者。
对 2012 年 1 月至 2018 年 5 月期间治疗的高危型肝母细胞瘤患儿进行回顾性研究。分析围手术期数据和长期预后。
52 例肝母细胞瘤患儿中,22 例被认为患有高危型肝母细胞瘤(8 名女孩和 14 名男孩)。诊断时的平均(标准差)年龄为 35(20)个月。这 22 例患儿中,5 例未经手术死亡。其余 17 例接受手术的患儿中,6 例行切除术(4 例右三叶切除术,2 例左三叶切除术),11 例行活体供肝移植术。中位随访时间为 48(12-90)个月。17 例患儿中有 13 例在最后一次随访时存活,4 例发生播散性疾病(3 例接受肝移植,1 例接受肝切除术)。整个高危型肝母细胞瘤患儿队列的 1、3 和 5 年总生存率分别为 77%、64%和 62%。接受手术的患儿中,移植组的 1、3 和 5 年生存率分别为 91%、82%和 73%,切除术组为 100%、83%和 83%。两组手术患儿的生存率无差异。
即使在资源有限的国家,高危型肝母细胞瘤的治疗也可取得良好效果。