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胸腺瘤中枢神经系统转移:来自 RYTHMIC 的真实世界观察简述报告。

Central Nervous System Metastases in Thymic Epithelial Tumors: A Brief Report of Real-World Insight From RYTHMIC.

机构信息

Thoracic Cancer Unit, Gustave Roussy, Villejuif, France.

Oscar Lambret, Lille, France.

出版信息

J Thorac Oncol. 2021 Dec;16(12):2144-2149. doi: 10.1016/j.jtho.2021.08.008. Epub 2021 Aug 26.

DOI:10.1016/j.jtho.2021.08.008
PMID:34455064
Abstract

Thymic epithelial tumors (TETs) are rare malignancies ranging from indolent thymoma A to aggressive thymic carcinomas (TCs). Brain metastases are extremely infrequent for TETs and have only been described in case reports or small single-center series. RYTHMIC (Réseau tumeurs THYMiques et Cancer) is a French nationwide network mandated to systematically review every TET case and prospectively includes all consecutive patients discussed by national or regional tumor boards. We analyzed patients with TETs and central nervous system (CNS) metastasis during their cancer history from this large French registry. In an 8-year period, 2909 patients were included in the database, including 248 TCs (8.5%). A total of 14 patients had CNS metastases, five (36%) at diagnosis and nine (64%) at relapse. Among them, 12 patients (86%) had a diagnosis of TC and two (14%) had thymoma A and B3. Surgical biopsies were performed, and the histologic subtype for non-TC tumors was centrally confirmed. Median overall survival was 22 months (95% confidence interval [CI]: 9.8-34.2), with longer, albeit not significant, overall survival when CNS metastases were present at diagnosis versus relapse (not reached versus 17 mo; p = 0.29); median progression-free survival was 13 versus 8 months (p = 0.06), respectively. A higher risk of death (hazard ratio = 5.34, 95% CI: 1.3-21.9, p = 0.02) and relapse (hazard ratio = 1.89, 95% CI: 0.9-3.7, p = 0.06) was observed for patients suffering from TC with brain metastases compared with those without CNS extension. CNS disease was extremely rare in our TET cohort (0.48%), reported at both diagnosis and progression, present primarily in TC, with prevalence rising to 4.9%.

摘要

胸腺上皮肿瘤(TETs)是罕见的恶性肿瘤,从惰性胸腺瘤 A 到侵袭性胸腺癌(TCs)不等。脑转移对于 TETs 来说极为罕见,仅在病例报告或小的单中心系列中有所描述。RYTHMIC(Réseau tumeurs THYMiques et Cancer)是一个法国全国性网络,负责系统审查每例 TET 病例,并前瞻性地包括国家或地区肿瘤委员会讨论的所有连续患者。我们分析了来自这个大型法国注册中心的 TET 患者及其癌症病史中的中枢神经系统(CNS)转移情况。在 8 年期间,该数据库共纳入 2909 例患者,其中包括 248 例 TC(8.5%)。共有 14 例患者发生 CNS 转移,其中 5 例(36%)在诊断时,9 例(64%)在复发时。其中,12 例(86%)患者诊断为 TC,2 例(14%)患者诊断为胸腺瘤 A 和 B3。进行了外科活检,非 TC 肿瘤的组织学亚型由中心确认。中位总生存期为 22 个月(95%置信区间[CI]:9.8-34.2),与复发时相比,CNS 转移在诊断时存在时总生存期较长(未达到 17 个月;p=0.29);中位无进展生存期分别为 13 个月和 8 个月(p=0.06)。与无脑膜转移的患者相比,患有脑转移的 TC 患者死亡(风险比=5.34,95%CI:1.3-21.9,p=0.02)和复发(风险比=1.89,95%CI:0.9-3.7,p=0.06)的风险更高。CNS 疾病在我们的 TET 队列中极为罕见(0.48%),在诊断和进展时均有报道,主要发生在 TC 中,患病率上升至 4.9%。

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