背景:非典型畸胎样/横纹肌样瘤很少见,成人高度恶性肿瘤,中位生存期为20个月。我们报告了一例接受脑室内化疗治疗的70岁女性的鞍状非典型畸胎瘤/横纹肌样瘤,其次是对鞍区AT/RT的当前管理的系统评价。
方法:在WebofScience上进行了全面系统的文献检索,Scopus,和PubMedCentral使用关键术语“鞍区”和“非典型畸胎瘤/横纹肌样瘤”,遵循系统审查和荟萃分析(PRISMA)指南的首选报告项目。数据,包括病人的人口统计,组织学,治疗,提取并分析总生存期。使用Kaplan-Meier生存曲线和对数秩分析来比较不同治疗方案之间的生存结果。
结果:我们的文献检索公开了123篇出版物。在预先指定的排除之后,从30份手稿中确定了41例鞍形AT/RT患者,和38个被包括在最终分析中。包括我们的病人,中位年龄为44岁(范围:20-70岁),女性占主导地位(94.7%).总的来说,与接受单一模式或不接受辅助治疗的患者相比,接受联合放化疗治疗的患者的总生存期显着增加(中位OS27vs.1.25个月;p=0.0052)。
结论:鞍区非典型畸胎瘤/横纹肌样瘤预后较差。辅助化疗和放疗与总生存率显著增加相关。早期考虑神经肿瘤学和放射肿瘤学转诊和管理可能对该患者人群有益。鞘内化疗是一种治疗方式,鉴于成人鞍区AT/RT的选择有限且目前预后不佳,需要进一步探索。
BACKGROUND: Atypical Teratoid/Rhabdoid tumors are rare, highly malignant tumors in adults, with a median survival of 20 months. We report a case of a sellar atypical teratoid/rhabdoid tumor in a 70-year-old female treated with intraventricular chemotherapy, followed by a systematic review of the current management of sellar AT/RTs.
METHODS: A comprehensive systematic literature search was conducted on Web of Science, Scopus, and PubMed Central using the key terms \"sellar\" and \"atypical teratoid/rhabdoid tumors\", following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Data, including patient demographics, histology, treatments, and overall survival were extracted and analyzed. Kaplan-Meier survival curves and log-rank analysis were used to compare survival outcomes between different treatment regimens.
RESULTS: Our literature search disclosed 123 publications. After prespecified exclusions, 41 patients with sellar AT/RT from 30 manuscripts were identified, and 38 were included in the final analysis. Including our patient, the median age was 44 (range: 20-70) with a substantial female predominance (94.7%). Collectively, patients who received combined chemoradiation therapy had a significantly increased overall survival compared to those who received single modality or no adjuvant therapies (median OS 27 vs. 1.25 months; p=0.0052).
CONCLUSIONS: Atypical teratoid/rhabdoid tumor in the sellar region carries a poor prognosis. Adjuvant chemotherapy and radiation therapy were associated with significantly increased overall survival. Early consideration of neuro-oncology and radiation-oncology referral and management is likely beneficial in this patient population. Intrathecal chemotherapy is a treatment modality that requires further exploration given the limited options and current dismal prognosis of adult sellar AT/RT.