摘要
背景/目的:回顾性评价和比较在非洲医院大约31年期间(1970-2001年)所有肝胚细胞瘤患者的临床特征、治疗方案、病理学检查及预后结果。方法:根据所给的治疗将40例肝胚细胞瘤的患者分成3个组。
Background/purpose The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all pa-tientswith hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001). Methods Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group Ⅰ (1970 to 1983, 14 patients) had no protocol therapy; group Ⅱ (1984 to 1988, 6 patients) received protocol treatment according to Children's Study Group (CCSG) guidelines; group Ⅲ (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed. Results Overall patient survival was as follows: group Ⅰ, 14%; group Ⅱ, 50%, and group Ⅲ, 80%. Deaths in group Ⅱ were caused by chemotherapy-induced immunosuppression only. Prognostic data for group Ⅲ showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P = .001) or vascular invasion (P = .001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperi-toneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival. Conclusions The introduction of protocol therapy has re- suited in a marked improvement in survival. Immunosup-pressionrelated sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.
