Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, China.
Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
Br J Neurosurg. 2024 Apr;38(2):425-432. doi: 10.1080/02688697.2021.1885620. Epub 2021 Feb 17.
Atypical teratoid/rhabdoid tumor (AT/RT) is well documented in children but less so in adults because of its rarity.
We report a series of five cases, a literature review and quantitative analysis of the survival outcome of adult AT/RT patients.
Seventy-four patients including our five cases (male: female = 16: 58) were evaluated, whose median age was 32.5 years (18-80 years). The commonest location was the sellar region. Median overall survival (OS) was 12.5 months (0.5-204.00 months). Chemotherapy was associated with OS (HR: 0.349, 95%CI: 0.176-0.694, = 0.003), while other factors did not influence OS. From Kaplan-Meier analysis, surgery combining postoperative chemotherapy and radiotherapy was associated with better prognosis (Log Rank: = 14.662, = 0.001).
Adult AT/RT is commoner in females and tends to recur rapidly after surgical resection. Chemotherapy may provide a survival benefit. Surgery combined with postoperative chemotherapy and radiotherapy was associated with better prognosis for adult AT/RT patients, though the overall prognosis was still poor.
虽然在儿童中已有充分的关于非典型畸胎样/横纹肌样肿瘤（AT/RT）的文献记载，但由于其罕见性，在成人中相关报道较少。
我们报道了 5 例病例，并进行了文献复习和对成人 AT/RT 患者生存结果的定量分析。
共评估了 74 例患者（包括我们的 5 例患者，男性：女性=16：58），中位年龄为 32.5 岁（18-80 岁）。最常见的部位是鞍区。中位总生存期（OS）为 12.5 个月（0.5-204.00 个月）。化疗与 OS 相关（HR：0.349，95%CI：0.176-0.694，P=0.003），而其他因素不影响 OS。从 Kaplan-Meier 分析来看，手术联合术后化疗和放疗与更好的预后相关（Log Rank：P=14.662，P=0.001）。
成人 AT/RT 以女性多见，且在手术切除后迅速复发。化疗可能会带来生存获益。手术联合术后化疗和放疗与成人 AT/RT 患者的预后相关，尽管总体预后仍较差。
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