背景:非典型畸胎样/横纹肌样(AT/RT)肿瘤是罕见且侵袭性的肿瘤,主要影响3岁以下的儿童。尽管积极治疗,儿科AT/RTs的总生存率仍然很低.由于它们的稀有性,关于预后因素知之甚少,也没有官方的治疗标准。
方法:按照PRISMA指南进行全面的数据库搜索。搜索词包括(非典型畸胎瘤样)和(非典型(畸胎瘤样或横纹肌样)肿瘤)。包括感兴趣的变量,但不仅限于,年龄,性别,肿瘤位置,治疗方式,切除范围,和总体生存率。
结果:共纳入936例患者的294项研究。该队列的中位年龄为22个月。与非手术治疗相比,接受手术治疗的患者的生存率存在显着差异(50.3个月与28个月,分别为;p<0.005)。有趣的是,切除程度并没有显着提高生存率(GTR的p=0.832,对于PR,p=0.650)。联合治疗与手术切除,放疗和化疗显示出最大的中位总生存期(54.9个月),并在多变量分析上显著提高了生存期(HR,0.48;95%CI,0.23-0.97;p=0.042)。
结论:这项研究的结果表明,虽然手术是小儿AT/RT的重要治疗方式,切除程度的影响尚不清楚.包括手术在内的多模式治疗,放射治疗,化疗可有效提高总生存率。未来的研究应该集中在利用更大的数据集来有效地解释混杂因素和偏见。
Atypical teratoid/rhabdoid tumors are rare and aggressive tumors that mainly affect children <3 years of age. Despite aggressive treatment, the overall survival rate for pediatric atypical teratoid/rhabdoid tumors remains poor. Due to their rarity, little is known regarding prognostic factors, and there is no official standard of treatment.
A comprehensive database search was conducted following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Search terms included \"atypical teratoid rhabdoid tumor\" and \"atypical (teratoid OR rhabdoid) tumor.\" Variables of interest included, but were not limited to, age, sex, tumor location, treatment modality, extent of resection, and overall survival.
The study included 294 articles and 936 patients. The median age of patients was 22 months. There was a significant difference in survival among patients receiving surgery compared with patients receiving nonoperative treatment (50.3 months vs. 28 months; P < 0.005). Extent of resection did not significantly improve survival (P = 0.832 for gross total resection, P = 0.650 for partial resection). Combination therapy with surgical resection, radiotherapy, and chemotherapy demonstrated the largest median overall survival (54.9 months) and significantly improved survival on multivariate analysis (hazard ratio, 0.48; 95% confidence interval, 0.23-0.97; P = 0.042).
The results of this study indicate that while surgery is a crucial treatment modality for pediatric atypical teratoid/rhabdoid tumors, the effect of extent of resection is unclear. Multimodal therapy including surgery, radiotherapy, and chemotherapy is effective in improving overall survival. Future studies should focus on using larger datasets to efficiently account for confounding factors and biases.