按级别预测中间分化的松果体实质肿瘤（PPTID）的生存率。
Prognosticating survival of pineal parenchymal tumors of intermediate differentiation (PPTID) by grade.
机构信息
Department of Neurological Surgery, University of Miami Miller School of Medicine, 1095 NW 14th Terrace, Miami, FL, 33136, USA.
出版信息
J Neurooncol. 2021 Nov;155(2):165-172. doi: 10.1007/s11060-021-03863-y. Epub 2021 Oct 4.
BACKGROUND
Pineal parenchymal tumors of intermediate differentiation (PPTID) are a rare group of pineal parenchymal tumors classified by histology as either World Health Organization (WHO) Grades 2 or 3. The rarity of these tumors in adults has left a number of clinical management questions open. Correspondingly, the aim of this study was to aggregate a large PPTID cohort with sufficient statistical power from a large national cancer database to analyze prognostic parameters.
METHODS
All PPTID patients aged over 18 years in the U.S. National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized and survival was modeled using Kaplan-Meier and Cox regression analyses.
RESULTS
A total of 103 adult PPTID patients were identified in the NCDB with 63 (61%) WHO Grade 2 and 40 (39%) WHO Grade 3 tumors. Overall, mean age was 53 ± 18 years with even gender distribution. A total of 75 (73%) patients underwent surgical resection for diagnosis, with gross total resection (GTR) was the most common resection outcome in 50/75 (67%). Chemotherapy was utilized in 18 (17%) patients, and radiation therapy in 37 (36%) patients. Overall, 5-year survival rate was estimated to be 54% (95% CI 42-64%), with mean survival was 84 (95% CI 69-99) months. Patients with Grade 2 tumors survived statistically longer than Grade 3 tumor counterparts (P < 0.01). Overall, older age (HR 1.09, P < 0.01) was associated with shorter survival, whereas GTR (HR 0.43, P = 0.02) was associated with longer survival. Both these parameters were significant within Grade 2 and Grade 3 subgroup analyses as well.
CONCLUSIONS
PPTID are rare tumors with expected mean survival more than 5 years, although Grade 2 tumors are expected to survive longer than Grade 3 tumors. Age and gross total resection are significant independent predictors of survival in PPTID overall, as well as within Grade 2 and Grade 3 subgroups separately. The prognostic role and benefit of adjuvant therapy is yet to be elucidated, mandating more molecular and biologic research be done to further optimize clinical management in the future.
背景
具有中等分化的松果体实质肿瘤（PPTID）是一组罕见的松果体实质肿瘤，根据组织学分类为世界卫生组织（WHO）分级 2 或 3。这些肿瘤在成年人中较为罕见，这使得许多临床管理问题悬而未决。因此，本研究的目的是从大型国家癌症数据库中汇集一个具有足够统计效力的大型 PPTID 队列，以分析预后参数。
方法
回顾性分析了美国国家癌症数据库（NCDB）中 2005 年至 2016 年间年龄超过 18 岁的所有 PPTID 患者。对数据进行总结，并使用 Kaplan-Meier 和 Cox 回归分析对生存率进行建模。
结果
NCDB 中共有 103 例成人 PPTID 患者，其中 63 例（61%）为 WHO 分级 2 级，40 例（39%）为 WHO 分级 3 级。总体而言，平均年龄为 53±18 岁，性别分布均匀。75 例（73%）患者接受手术切除诊断，最常见的切除结果是 50/75（67%）例大体全切除（GTR）。18 例（17%）患者接受化疗，37 例（36%）患者接受放疗。总的 5 年生存率估计为 54%（95%CI 42-64%），平均生存时间为 84（95%CI 69-99）个月。WHO 分级 2 级肿瘤患者的生存时间明显长于 WHO 分级 3 级肿瘤患者（P<0.01）。总的来说，年龄较大（HR 1.09，P<0.01）与生存时间较短相关，而 GTR（HR 0.43，P=0.02）与生存时间较长相关。这两个参数在 WHO 分级 2 级和 3 级亚组分析中也是显著的。
结论
PPTID 是罕见的肿瘤，预期平均生存时间超过 5 年，尽管 WHO 分级 2 级肿瘤的预期生存时间长于 WHO 分级 3 级肿瘤。年龄和大体全切除是 PPTID 总体以及 WHO 分级 2 级和 3 级亚组中生存的独立显著预测因素。辅助治疗的预后作用和益处仍有待阐明，需要进行更多的分子和生物学研究，以在未来进一步优化临床管理。
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