Pediatric choroid plexus papilloma arising from the cerebellopontine angle: systematic review with illustrative case
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ORIGINAL ARTICLE
Pediatric choroid plexus papilloma arising from the cerebellopontine angle: systematic review with illustrative case Mairre James S. Gaddi 1 & Jeffrey I. Lappay 2 & Kevin Ivan P. Chan 1 & Juan Silvestre G. Pascual 1 & Alaric Emmanuel M. Salonga 1 Received: 9 June 2020 / Accepted: 22 September 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords Choroid plexus papilloma . Cerebellopontine angle . Pediatric . Children . Adolescent
Introduction Choroid plexus tumors are uncommon intraventricular tumors that develop from the choroid plexus of the central nervous system [1]. They account for 0.3–0.8% of all brain tumors overall and 2–5% of all pediatric brain tumors [2, 3]. According to the World Health Organization classification of central nervous system tumors, they are classified either
* Mairre James S. Gaddi [email protected] 1
Division of Neurosurgery, Department of Neurosciences, University of the Philippines - Philippine General Hospital, Manila, Philippines
2
Division of Pediatric Neurology, Department of Neurosciences and Pediatrics, University of the Philippines - Philippine General Hospital, Manila, Philippines
into choroid plexus papilloma (CPP) (WHO Grade 1), atypical choroid plexus papilloma (WHO Grade 2), or choroid plexus carcinoma (WHO Grade 3) [2].
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