Clinicopathological characteristics and treatment of hepatoblastoma with macrotrabecular structures
10.3760/cma.j.cn113884-20200215-00067
- VernacularTitle:包含巨小梁结构肝母细胞瘤临床病理特点及治疗体会
- Author:
Xiaofeng CHANG
1
;
Wei YANG
;
Qinghua REN
;
Hong QIN
;
Huanmin WANG
;
Deguang MENG
;
Huaida TENG
Author Information
1. 国家儿童医学中心 首都医科大学附属北京儿童医院肿瘤外科，北京 100045
- From:
Chinese Journal of Hepatobiliary Surgery
2020;26(6):435-438
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To analyze the clinicopathological characteristics and treatment strategies of hepatoblastoma with macrotrabecular structures.Methods:To retrospectively analyze the data of children with hepatoblastoma treated in the Department of Oncology at Beijing Children's Hospital affiliated to Capital Medical University amd Baoding Children's Hospital from January 1, 2011 to December 31, 2019. The study structure consisted of collecting clinical data and formulating treatment plan, including clinical stage, alpha-fetoprotein (AFP), chemotherapy plan, surgical margin, pathological classification and follow-up data to study the long-term prognosis of these patients.Results:Among 17 patients, 13 males and 4 females, age 5 to 134 months; 5 patients had the macrotrabecular type, 10 patients had the epithelial type with macrotrabecular, 2 patients had the mixed epithelial and mesenchymal with macrotrabecular type. For the PRETEXT staging, 1 patient had stage I, 2 patients had stage II, 14 patients had stage III and IV. A total of 15 patients received preoperative chemotherapy, and 6 achieved partial response. Among 17 patients, 10 had negative resection margins. The AFP of 7 patients was normal after chemotherapy, and 10 patients relapsed after surgery. The 2-year event-free survival was 26.18%.Conclusions:Patients with hepatoblastoma containing giant trabecular components are very rare. Among them, the recurrence rate of patients with giant trabecular and epithelial and giant trabecular components was high. Preoperative neoadjuvant chemotherapy (vincristine + irinotecan), transcatheter arterial chemoembolization and liver transplantation are treatment options for this type of hepatoblastoma.
