University of Regensburg, Regensburg, Germany.
Oncology Development, AbbVie, Chicago, IL, USA.
J Neurooncol. 2022 Feb;156(3):599-613. doi: 10.1007/s11060-021-03942-0. Epub 2022 Jan 8.
Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established.
CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled.
For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators.
Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.
针对脉络丛肿瘤（CPT）的化疗标准尚未确立。
CPT-SIOP-2000（NCT00500890）是一项针对所有 CPT 的国际注册研究，其中包括针对高危 CPT 的卡铂/依托泊苷/长春新碱（CarbEV）与环磷酰胺/依托泊苷/长春新碱（CycEV）化疗的随机分组。对于年龄大于 3 岁的患者，建议进行放疗：对于非转移性 CPC、未完全切除的非典型脉络丛乳头状瘤（APP）或转移性脉络丛乳头状瘤（CPP），采用局部野照射；对于转移性 CPC/APP 和对治疗无反应的 CPC，采用颅脊髓野照射。高危定义为脉络丛癌（CPC）、未完全切除的 APP 和所有转移性 CPT。从 2000 年到 2010 年，来自 23 个国家的 158 名 CPT 患者入组该研究。
对于随机分组的 CPC 患者，接受 CarbEV（n=20）治疗的患者的 5/10 年无进展生存率（PFS）分别为 62%/47%，而接受 CycEV（n=15）治疗的患者为 27%/18%（意向治疗，HR 2.6，p=0.032）。在该注册研究中，组织学分级是最具影响力的预后因素：对于 CPP（n=55），5/10 年总生存率（OS）和无事件生存率（EFS）概率分别为 100%/97%和 92%/92%；对于 APP（n=49），分别为 96%/96%和 76%/76%；对于 CPC（n=54），分别为 65%/51%和 41%/39%。33 例年龄小于 3 岁的 CPC 患者中，12 例在中位随访 8.52 年后仍存活。手术范围和转移并非独立的预后因素。
脉络丛癌的化疗是可行且有效的。CarbEV 优于 CycEV。部分 CPC 无需放疗即可治愈。
