Prognosticating survival of pineal parenchymal tumors of intermediate differentiation (PPTID) by grade
Prognosticating survival of pineal parenchymal tumors of intermediate differentiation (PPTID) by grade
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DOI：
10.1007/s11060-021-03863-y
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发表时间：
2021-10-04
影响因子：
3.9
通讯作者：
Ivan, Michael E.
中科院分区：
文献类型：
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作者：
Lu, Victor M.;Luther, Evan M.;Ivan, Michael E.
背景：中分化松果体实质肿瘤（PPTID）是一组罕见的松果体实质肿瘤，组织学分类为世界卫生组织（WHO）2级或3级。这些肿瘤在成人中的罕见性留下了许多临床管理问题。相应地，本研究的目的是从一个大型国家癌症数据库中聚集一个具有足够统计能力的大型PPTID队列，以分析预后参数。方法回顾性分析2005年至2016年美国国家癌症数据库（NCDB）中所有年龄超过18岁的PPTID患者。数据进行了总结，生存率采用Kaplan-Meier和考克斯回归analysis.Results共103例成人PPTID患者被确定在NCDB与63（61%）WHO 2级和40（39%）WHO 3级肿瘤。总体而言，平均年龄为53 ± 18岁，性别分布均匀。共有75例（73%）患者接受了手术切除诊断，其中50/75例（67%）患者的大体全切除（GTR）是最常见的切除结局。18例（17%）患者接受化疗，37例（36%）患者接受放疗。总体而言，5年生存率估计为54%（95% CI 42-64%），平均生存期为84（95% CI 69-99）个月。2级肿瘤患者的生存时间明显长于3级肿瘤患者（P < 0.01）。总体而言，年龄较大（HR 1.09，P < 0.01）与生存期较短相关，而GTR（HR 0.43，P = 0.02）与生存期较长相关。这两个参数都是显着的2级和3级亚组analysis.Conclusions PPTID是罕见的肿瘤，预计平均生存期超过5年，虽然2级肿瘤预计生存时间长于3级肿瘤。年龄和大体全切除是PPTID总体生存率的重要独立预测因素，2级和3级亚组也是如此。辅助治疗的预后作用和益处尚未阐明，需要进行更多的分子和生物学研究，以进一步优化未来的临床管理。
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.