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

Abstract

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.

Final results of the Choroid Plexus Tumor study CPT-SIOP-2000

“…Treatment recommendations for CPT include multidisciplinary approaches, with maximal surgical resection for all CPT [8][9][10][11][12][13][14], followed by chemotherapy [11,[15][16][17][18][19][20][21] and radiotherapy [22][23][24] for high-risk CPT. The prognosis of CPC remains dismal when tumor resection is the only treatment modality, and the role, sequence, and intensity of primary chemotherapy remain debatable [13,19,21].…”

Posterior fossa choroidplexus papilloma in the pediatric population: case series and literature review

“…The results of the literature review are detailed in Table 1 , along with those of the present case series. 2 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 …”

Infratentorial choroid plexus tumors in children: Case report and review of literature

“…Unlike supratentorial lesions (which are known to be hypervascularized tumors), infratentorial CPTs are less vascularized, especially those with an extraventricular location. (9,8) This fact justifies a lower rate of hemorrhagic complications in posterior fossa CPT. (9,8) In the case operated by our team, the lesion was not hypervascularized and there was no technical difficulty in bleeding control.…”

“…(9,8) This fact justifies a lower rate of hemorrhagic complications in posterior fossa CPT. (9,8) In the case operated by our team, the lesion was not hypervascularized and there was no technical difficulty in bleeding control.…”

“…Previous e214202 reports discuss that this difference is due to the lower obstructive component of CPA lesions and lower CSF production, as they are less vascularized tumors. (6,8,18,19) As complete tumor resection is achieved in most cases (with resolution of obstructive factors and overproduction of CSF), only 27% of cases remains shunt dependent and most of them hydrocephalus persists due to disabsorptive problems. (8,16) Regarding the best treatment for hydrocephalus and the choice between external ventricular drainage (EVD), endoscopic third ventriculostomy (ETV) or VPS is still controversial.…”