Intraventricular choroid plexus tumors: clinical characteristics and impact of current management on survival

J Neurooncol. 2020 Sep;149(2):283-292. doi: 10.1007/s11060-020-03603-8. Epub 2020 Sep 8.


Introduction: Choroid plexus tumors (CPTs) represent one of the most common intraventricular tumors. Although most are benign, they often reach considerable sizes before clinical manifestation, challenging their surgical management. We aim to describe the clinical characteristics and the impact of current management on the survival of patients harboring intraventricular CPT.

Methods: The National Cancer Database (NCDB) was queried to identify biopsy-proven intraventricular CPT patients (2004-2015). Demographic and patterns of care were described, the log-rank method was used to independently analyze survival according to age, WHO grade and extent of resection (EOR). Multivariate analysis was performed to investigate the impact of prognostic factors on overall survival (OS).

Results: A total of 439 CPT patients with known WHO grade were included. WHO grade I tumors were more frequent in adults, while WHO grade III tumors were more common in pediatric population. Most CPTs were benign, with a median tumor size of 3-4 cm. Mean tumor size in pediatric population was greater than in adult population (4.39 cm vs. 2.7 cm; p < 0.01). Frequency was similar between males and females (51.7% vs. 48.3%; p > 0.0.5). Five- and ten-year OS among all patients was 87% and 84%, respectively. EOR was not associated with survival for any WHO grade. On multivariable analysis, only patient age (p = 0.022), WHO grade (p = 0.003) and medical comorbidity scores (p = 0.002) were independently associated with OS after diagnosis.

Conclusion: Patients with CPTs present at different stages of life, with sizable tumor burden and distinct WHO grade prevalence. Considering their favorable survival, efforts to improve tumor control should be meticulously weighed against the long-term risk associated with surgery, radiation, and chemotherapy.

Keywords: Atypical choroid plexus papilloma; Choroid plexus carcinoma; Choroid plexus papilloma; Epidemiology; Prognostic.

MeSH terms

  • Adolescent
  • Adult
  • Cerebral Ventricle Neoplasms / mortality*
  • Cerebral Ventricle Neoplasms / pathology
  • Cerebral Ventricle Neoplasms / therapy
  • Child
  • Child, Preschool
  • Choroid Plexus Neoplasms / mortality*
  • Choroid Plexus Neoplasms / pathology
  • Choroid Plexus Neoplasms / therapy
  • Combined Modality Therapy
  • Databases, Factual
  • Disease Management
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Survival Rate
  • Young Adult