Final Results of the Choroid Plexus Tumor Study CPT-SIOP-2000

Authors

Johannes E Wolff
Stefaan W Van Gool
Tezer Kutluk
Blanca Diez
Rejin Kebudi
Beate Timmermann
Miklos Garami
Jaroslav Sterba
Gregory N Fuller
Brigitte Bison
Uwe R Kordes

Publication Date

2-1-2022

Journal

Journal of Neuro-Oncology

Abstract

INTRODUCTION: Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established.

METHODS: 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.

RESULTS: 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.

CONCLUSIONS: Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.

Keywords

Antineoplastic Combined Chemotherapy Protocols, Carcinoma, Choroid Plexus Neoplasms, Etoposide, Humans, Randomized Controlled Trials as Topic, Registries, Treatment Outcome, Vincristine

DOI

10.1007/s11060-021-03942-0

PMID

34997889

PMCID

PMC8860833

PubMedCentral® Posted Date

1-8-2022

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes