ArticleJournal of neuro-oncology2022
Final results of the Choroid Plexus Tumor study CPT-SIOP-2000.
Article in Journal of neuro-oncology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. It is linked to trial NCT00500890 (A Pilot Study Evaluating the Feasibility of an Intercontinental Phase III Chemotherapy Study for Patients With Choroid Plexus Tumors), which is not on this map. Cited by 24 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
A Pilot Study Evaluating the Feasibility of an Intercontinental Phase III Chemotherapy Study for Patients With Choroid Plexus Tumors
Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 29 citations in OpenAlex.
- Risk Factors for Delayed Leptomeningeal Dissemination in Choroid Plexus Papillomas: A Systematic Review and Illustrative Case.Current oncology (Toronto, Ont.) · 2026Pooled it
- Strategic roadmap for delivery of clinical trials in rare childhood central nervous system (CNS) tumours: a multi-stakeholder consensus.British journal of cancer · 2026Article
- Overactivation of SHH signaling induces a cascade of dedifferentiation and oncogenesis in a new mouse model for choroid plexus carcinoma.Acta neuropathologica communications · 2026Article
- Choroid plexus carcinoma: state of the field and emerging directions.Oncogenesis · 2026Review
- Report from the National Pediatric Cancer Foundation - infantile glioma and other non-embryonal central nervous system tumors: evolving molecular advances and current treatment landscape.Frontiers in oncology · 2026Review
- CPT-SIOP registry: evaluation of risk factors for disease progression in pediatric choroid plexus papilloma.Journal of neuro-oncology · 2025Article
- Primary pigmented papillary epithelial tumor of the sella: case report and literature review.Brain tumor pathology · 2025Review
- SOX2 commands LIM homeobox transcription factors in choroid plexus development and tumorigenesis.Neuro-oncology · 2025Article
- Activation of Wnt/β-catenin signaling is critical for the tumorigenesis of choroid plexus.Neuro-oncology · 2025Article
- Leptomeningeal Dissemination in Choroid Plexus Tumors: Magnetic Resonance Imaging Appearance and Risk Factors.Children (Basel, Switzerland) · 2025Article
- Developing a nomogram based on SEER database for predicting prognosis in choroid plexus tumors.Scientific reports · 2024Article
- Article
- Pediatric-Like Brain Tumors in Adults.Advances and technical standards in neurosurgery · 2024Review
- Metastatic choroid plexus papilloma presenting as a sellar mass: A case report and literature review.Surgical neurology international · 2024Article
- Exoscopic removal of the fourth ventricle choroid plexus papilloma with use of a midline suboccipital osteoplastic craniotomy.Neurosurgical focus: Video · 2024Article
- Approaches to Minimise the Neurodevelopmental Impact of Choroid Plexus Carcinoma and Its Treatment.Life (Basel, Switzerland) · 2023Review
- Distinct challenge: the case report of disseminated adult atypical choroid plexus papilloma.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2023Article
- Cancer Stem Cells in Tumours of the Central Nervous System in Children: A Comprehensive Review.Cancers · 2023Review
- Molecular heterogeneity of pediatric choroid plexus carcinomas determines the distinctions in clinical course and prognosis.Neuro-oncology · 2023Article
- Leptomeningeal dissemination in pediatric brain tumors.Neoplasia (New York, N.Y.) · 2023Article
Corrections and comments
- Erratum issued
Authors and funding
11 authors at 11 institutions in 6 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionStandards for chemotherapy against choroid plexus tumors (CPT) have not yet been established.
methodsCPT-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.
resultsFor 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.
conclusionsChemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.
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