Pediatric choroid plexus papilloma arising from the cerebellopontine angle: systematic review with illustrative case

Childs Nerv Syst. 2021 Mar;37(3):799-807. doi: 10.1007/s00381-020-04896-w. Epub 2020 Sep 26.


Introduction: Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system. Choroid plexus papillomas arising from the cerebellopontine angle have been reported to almost exclusively occur in adults and are rarely found in children.

Methods: We report a systematic review conducted in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines of SCOPUS and PubMed databases for case reports and case series of choroid plexus papillomas arising in the cerebellopontine angle in the pediatric population and discuss clinical presentation, imaging features, management options, and outcomes. We also report a case managed at our center.

Results: Ten cases of pediatric choroid plexus papillomas arising in the cerebellopontine angle were identified from the systematic review in addition to the case reported here, resulting in a total of eleven cases. The patients' median age was 8 years with a slight female sex predilection (1.2:1). Patients most commonly presented with headache, cerebellar signs, and cranial nerve palsies with median duration of symptoms at 4 months. All patients underwent surgical treatment with majority achieving gross total excision. No deaths were reported at median follow-up of 12 months. Complete neurologic recovery was attained in seven cases while partial recovery was seen in two cases.

Conclusion: Choroid plexus papillomas found in the cerebellopontine angle in the pediatric population are extremely rare but they should be considered in the differential diagnosis. Complete surgical resection is the mainstay of treatment with excellent outcomes achievable in majority of patients.

Keywords: Adolescent; Cerebellopontine angle; Children; Choroid plexus papilloma; Pediatric.

Publication types

  • Systematic Review

MeSH terms

  • Adult
  • Cerebellopontine Angle / diagnostic imaging
  • Cerebellopontine Angle / surgery
  • Cerebral Ventricle Neoplasms*
  • Child
  • Choroid Plexus
  • Choroid Plexus Neoplasms* / diagnostic imaging
  • Choroid Plexus Neoplasms* / surgery
  • Female
  • Humans
  • Papilloma, Choroid Plexus* / diagnostic imaging
  • Papilloma, Choroid Plexus* / surgery
  • Supratentorial Neoplasms*