Background:Choroid plexus tumors (CPTs) are rare neoplasms found in the central nervous system, comprising 1% of all brain tumors. These tumors include choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). Although gross total resection for choroid plexus papillomas (CPPs) is associated with long-term survival, there is a scarcity of prospective data concerning the role and sequence of neoadjuvant therapy in treating aCPP and CPC.Methods:From the years 2000 to 2019, 679 patients with CPT were identified from the Surveillance, Epidemiology, and End Result (SEER) database. Among these patients, 456 patients had CPP, 75 patients had aCPP, and 142 patients had CPC. Univariate and multivariable Cox proportional hazard models were run to identify variables that had a significant impact on the primary endpoint of overall survival (OS). A predictive nomogram was built for patients with CPC to predict 5-year and 10-year survival probability.Results:Histology was a significant predictor of OS, with 5-year OS rates of 90, 79, and 61% for CPP, aCPP, and CPC, respectively. Older age and African American race were prognostic for worse OS for patients with CPP. Older age was also associated with reduced OS for patients with aCPP. American Indian/Alaskan Native race was linked to poorer OS for patients with CPC. Overall, treatment with gross total resection or subtotal resection had no difference in OS in patients with CPP or aCPP. Meanwhile, in patients with CPC, gross total resection (GTR) was associated with significantly better OS than subtotal resection (STR) only. However, there is no difference in OS between patients that receive GTR and patients that receive STR with adjuvant therapy. The nomogram for CPC considers types of treatments received. It demonstrates acceptable accuracy in estimating survival probability at 5-year and 10-year intervals, with a C-index of 0.608 (95% CI of 0.446 to 0.77).Conclusions:This is the largest study on CPT to date and highlights the optimal treatment strategies for these rare tumors. Overall, there is no difference in OS with GTR vs. STR in CPP or aCPP. Furthermore, OS is equivalent for CPC with GTR and STR plus adjuvant therapy.
背景：脉络丛肿瘤（CPTs）是中枢神经系统中罕见的肿瘤，占所有脑肿瘤的1%。这些肿瘤包括脉络丛乳头状瘤（CPP）、非典型脉络丛乳头状瘤（aCPP）和脉络丛癌（CPC）。尽管脉络丛乳头状瘤（CPPs）的肉眼全切除与长期生存相关，但关于新辅助治疗在治疗aCPP和CPC中的作用和顺序的前瞻性数据仍然匮乏。 方法：从2000年至2019年，从监测、流行病学和最终结果（SEER）数据库中识别出679例CPT患者。在这些患者中，456例为CPP，75例为aCPP，142例为CPC。采用单变量和多变量Cox比例风险模型来确定对总生存期（OS）这一主要终点有显著影响的变量。为CPC患者构建了一个预测列线图，以预测5年和10年生存概率。 结果：组织学是OS的重要预测因子，CPP、aCPP和CPC的5年OS率分别为90%、79%和61%。年龄较大和非裔美国人种是CPP患者OS较差的预后因素。年龄较大也与aCPP患者的OS降低相关。美洲印第安人/阿拉斯加原住民种族与CPC患者较差的OS相关。总体而言，对于CPP或aCPP患者，肉眼全切除或次全切除治疗在OS上没有差异。同时，在CPC患者中，仅肉眼全切除（GTR）与次全切除（STR）相比，OS显著更好。然而，接受GTR的患者与接受STR加辅助治疗的患者在OS上没有差异。CPC的列线图考虑了所接受的治疗类型。它在估计5年和10年生存概率方面显示出可接受的准确性，C指数为0.608（95% CI为0.446至0.77）。 结论：这是迄今为止关于CPT的最大规模研究，并强调了这些罕见肿瘤的最佳治疗策略。总体而言，对于CPP或aCPP，GTR与STR在OS上没有差异。此外，对于CPC，GTR与STR加辅助治疗的OS是相当的。
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