Bahar Michal, Hashem Hasan, Tekautz Tanya, Worley Sarah, Tang Anne, de Blank Peter, Wolff Johannes
Department of Pediatric Hematology Oncology & Bone Marrow Transplantation, Cleveland Clinic Children's, S20 Cleveland Clinic Foundation 9500 Euclid Ave, Cleveland, OH, 44195, USA.
Department of Pediatric Hematology Oncology, University Hospitals Rainbow Babies and Children's Hospital, Cleveland, OH, USA.
J Neurooncol. 2017 May;132(3):427-432. doi: 10.1007/s11060-017-2384-1. Epub 2017 Mar 13.
Choroid plexus tumors (CPT) are rare neoplasms accounting for 1-4% of all pediatric brain tumors. They are divided into choroid plexus papilloma (CPP), atypical choroid plexus papilloma (APP) and choroid plexus carcinoma (CPC). CPTs are known to primarily affect children less than 2 years of age. Gross total resection is the most important predictor of survival especially in CPC. Although small case series have been published, limited clinical data are available to describe treatment and outcome of CPTs. More clinical data would be necessary to complete the picture, particularly in populations that are not age limited. Here we share data from the two major hospitals in Cleveland to describe treatment and outcome of adult and pediatric patients. We performed a retrospective analysis of patients with CPT seen in Cleveland Clinic from 1990 to 2015 and at University Hospitals from 1994 to 2015. Results were compared to previously published historical controls. We identified 30 cases with CPT, including 22 pediatric and eight adult cases; 11 females and 19 males. The mean age at presentation was 12.4 years with a median age of 4.5 years (range 2 months-51 years). Gross total surgical resection was achieved in 22, subtotal resection in four, partial resection in two and unknown in two. The histology was CPP in 23 patients, two of whom developed recurrence requiring repeat resection and adjuvant therapy. Median event free survival (EFS) for CPP patients was 7.6 years. The histology was CPC in seven patients. All CPC patients were treated with adjuvant therapy. Median EFS of CPC patients was 4.4 years. Overall survival of all CPT patients was 100% with a median follow up of 7 years. A systematic literature review identified 1012 CPT patients treated from 1989 to 2013. The mean and median age of CPT patients was 13 and 3 years respectively. The median survival of 541 CPP patients was undefined vs. 2.7 years for the 452 CPC patients. The difference between the two populations was highly significant (p < 0.001). Kaplan-Meier survival curves comparing CPTs at Cleveland Clinic and University Hospitals versus a systematic literature review showed a statistically significant advancement in overall survival among the patients treated at Cleveland Clinic and University Hospitals. Our data are consistent with the literature review regarding epidemiology, clinical presentation, and treatment modalities but differed in regards to survival. Differences in survival may be related to different methods of data collection or details in patient care.
脉络丛肿瘤（CPT）是罕见肿瘤，占所有儿童脑肿瘤的1%-4%。它们分为脉络丛乳头状瘤（CPP）、非典型脉络丛乳头状瘤（APP）和脉络丛癌（CPC）。已知CPT主要影响2岁以下儿童。手术全切是生存的最重要预测因素，尤其是在CPC中。尽管已发表了一些小病例系列，但描述CPT治疗及预后的临床数据有限。需要更多临床数据才能全面了解情况，特别是在无年龄限制的人群中。在此，我们分享来自克利夫兰两家主要医院的数据，以描述成人和儿童患者的治疗及预后。我们对1990年至2015年在克利夫兰诊所以及1994年至2015年在大学医院就诊的CPT患者进行了回顾性分析。将结果与之前发表的历史对照进行比较。我们共确定了30例CPT患者，包括22例儿童患者和8例成人患者；11例女性和19例男性。就诊时的平均年龄为12.4岁，中位年龄为4.5岁（范围为2个月至51岁）。22例实现了手术全切，4例次全切，2例部分切除，2例情况不明。23例患者的组织学类型为CPP，其中2例复发，需要再次切除并接受辅助治疗。CPP患者的无事件生存期（EFS）中位数为7.6年。7例患者的组织学类型为CPC。所有CPC患者均接受了辅助治疗。CPC患者的EFS中位数为4.4年。所有CPT患者的总生存率为100%，中位随访时间为7年。一项系统文献综述确定了1989年至2013年期间接受治疗的1012例CPT患者。CPT患者的平均年龄和中位年龄分别为13岁和3岁。541例CPP患者的中位生存期未明确，而452例CPC患者的中位生存期为2.7年。这两组人群之间的差异非常显著（p＜0.001）。比较克利夫兰诊所和大学医院的CPT患者与系统文献综述的Kaplan-Meier生存曲线显示，在克利夫兰诊所和大学医院接受治疗的患者总体生存率有统计学意义的提高。我们的数据在流行病学、临床表现和治疗方式方面与文献综述一致，但在生存率方面有所不同。生存率的差异可能与不同的数据收集方法或患者护理细节有关。
