背景:成人鞍区非典型畸胎样/横纹肌样瘤(AT/RT)是一种罕见的病变。我们的目的是阐明临床,放射学,和病理特征,治疗策略,以及这种疾病的结果。方法:回顾性分析2015年1月至2018年12月5例成年鞍区AT/RT患者的临床资料。此外,我们对成人鞍区AT/RT的报告数据进行了回顾.结果:患者(n=5)为女性,中位年龄为50岁。症状的平均持续时间,其中头痛最常见,为1.6个月(范围,2周-8个月)。平均肿瘤大小为2.82cm(范围,1.9-4.5厘米)。所有病灶形状不规则。MRI显示五个病变中的三个不均匀增强。5例患者中有4例接受了次全切除术(STR)和1例全全切除术(GTR)。然而,一名患者接受术后辅助放疗,1例患者接受了术后放化疗联合治疗.对报告数据的审查显示,已报告了39例成年鞍区AT/RT。估计的中位总生存期(OS)为23个月,1年生存率估计为59.7%。GTR患者的中位OS为28个月,STR患者为17个月。Kaplan-Meier分析显示,MIB-1/Ki67指标值高(≥35%)的患者与指标值低(<35%)的患者相比,OS明显较短(p=0.033),接受术后联合放化疗的患者的OS比未接受放化疗的患者长(p<0.001)。结论:成人鞍区AT/RT是一种生长迅速的肿瘤,预后较差。组织学上高水平的MIB1/Ki-67可能表明肿瘤的侵袭性特征。最大安全切除后辅助放疗联合化疗可能是成人鞍区AT/RT的最佳治疗策略。
Background: Adult sellar region atypical teratoid/rhabdoid tumor (AT/RT) is a rare lesion. We aimed to elucidate clinical, radiologic, and pathological characteristics, treatment strategies, and outcomes of this disease. Methods: Five adult sellar AT/RT patients were retrospectively analyzed between January 2015 and December 2018. In addition, we performed a
review of the reported data on adult sellar AT/RT. Results: Patients (n = 5) were female with a median age of 50 years. The mean duration of symptoms, of which headache was the most frequent, was 1.6 months (range, 2 weeks-8 months). The average tumor size was 2.82 cm (range, 1.9-4.5 cm). All lesions were irregularly shaped. MRI showed heterogeneous enhancement in three of five lesions. Four of five patients underwent subtotal resection (STR) and one gross total resection (GTR). Whereas, one patient received post-operative adjuvant radiotherapy, one patient received post-operative combination of radio- and chemotherapy. The
review of the reported data showed that 39 cases of adult sellar AT/RT had been reported. The estimated median overall survival (OS) was 23 months with a 1-year survival estimate of 59.7%. The median OS for patients with GTR was 28 months and 17 months for patients with STR. Kaplan-Meier analysis showed that patients with high (≥35%) MIB-1/Ki67 index value had a significantly shorter OS compared with those with low (<35%) index value (p = 0.033), and that patients who received post-operative combination radio- and chemotherapy had longer OS than that of those who did not (p < 0.001). Conclusion: Adult sellar region AT/RT is a rapidly growing tumor with a poor prognosis. High levels of MIB1/Ki-67 on histology may indicate aggressive feature of the tumor. Maximal safe resection followed by adjuvant radiotherapy combined with chemotherapy may be the optimal therapeutic strategy for adult sellar region AT/RT.