Choroid Plexus Tumor Management and Molecular Characterization

Choroid plexus tumours (CPTs) are rare intraventricular neoplasms that range from benign papillomas to highly aggressive carcinomas. Management traditionally centres on maximal safe surgical resection, with gross total resection associated with the best long-term outcomes. In cases of incomplete excision or high-grade histology, adjuvant therapies including chemotherapy and radiotherapy are considered, though their sequencing and dosage remain areas of active investigation. Prognostic models, notably nomograms derived from large population databases, now inform risk stratification and guide individualised treatment decisions. At the molecular level, recent studies have employed multiomics approaches—integrating genomic, transcriptomic and epigenomic data—to distinguish choroid plexus papilloma from carcinoma, revealing distinct mutational landscapes (for example in TP53, EPHA7) and methylation signatures. These findings offer potential biomarkers for diagnosis and targets for future therapies. Epigenetic profiling has further delineated subgroups with differing risks of recurrence, prompting a shift towards precision medicine in CPT care.

Research from Nature Portfolio

A large registry-based study developed and validated a prognostic nomogram for patients with choroid plexus tumours using surveillance data spanning two decades. Independent prognostic factors identified include age, tumour size, histopathological subtype and treatment modalities. The resulting tool demonstrated high discrimination for 5-, 10- and 15-year survival probabilities and good calibration in external testing, supporting its use in clinical decision-making and patient counselling.

Choroid Plexus Tumor Management and Molecular Characterization publication trend

The graph below shows the total number of articles in choroid plexus tumor management and molecular characterization across all publications each year (not limited to Nature Index journals).

Technical terms

Choroid plexus tumour: A neoplasm arising from the epithelium of the brain’s choroid plexus, classified by WHO as papilloma (grade I), atypical papilloma (grade II) or carcinoma (grade III).

Gross total resection (GTR): Surgical removal of all visible tumour tissue, typically associated with improved survival in CPTs.

Nomogram: A statistical tool that integrates multiple prognostic variables to estimate individual patient outcomes, often presented in a graphical format.

DNA methylation: An epigenetic modification involving addition of methyl groups to cytosine residues, used to classify tumour subgroups and predict clinical behaviour.

Epigenomic profiling: Comprehensive analysis of epigenetic marks—such as methylation—across the genome to identify regulatory differences between tumour types.

References

1. Developing a nomogram based on SEER database for predicting prognosis in choroid plexus tumors. Scientific Reports (2024).
2. Comprehensive multiomics analysis reveals distinct differences between pediatric choroid plexus papilloma and carcinoma. Acta Neuropathologica Communications (2024).
3. Prognostic Factors and Nomogram for Choroid Plexus Tumors: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis. Cancers (2024).
4. DNA methylation signature is prognostic of choroid plexus tumor aggressiveness. Clinical Epigenetics (2019).
5. Final results of the Choroid Plexus Tumor study CPT-SIOP-2000. Journal of Neuro-Oncology (2022).