Choroid Plexus Papilloma with Dandy Walker Variant: Co-existence or Association- A Case Report

Abstract

Choroid plexus tumors are rare intracranial tumors which account for 0.4-0.6% of all brain tumors. Choroid plexus tumors represent a spectrum of neoplasms derived from papillary epithelium of normal choroid plexus, including well-differentiated papilloma (WHO grade I), intermediate form as atypical Choroid Plexus Papilloma (WHO grade II) and highly aggressive choroid plexus carcinomas (WHO grade III). Though rare, it is responsible for the communicating hydrocephalus in children due to overproduction of cerebrospinal fluid. Due to advances in molecular biology and better understanding of the tumorigenesis of choroid plexus papilloma, now it is established that several genetic syndromes and central nervous sytem abnormalities are associated with this tumor. Here, we reported a case of a 10 months old child who presented with sudden deterioration of consciousness level and after thorough evaluation, diagnosed as a case of Choroid Plexus Papilloma with Dandy Walker Variant. Till date, this is the first reported case of the association/ co-existence of such two conditions which needs further evaluation.
 Bangladesh Med J. 2017 May; 46 (2): 61-65

A rare combination of a choroid plexus papilloma and Dandy-Walker variant

“…Her occipito-frontal circumference (OFC) at birth was 33cm and her length was 51cm. At 4 months of age, parents noticed that there was a lag in achieving developmental milestones, as there was no head control, though they did not seek medical ___________________________________________ 1 Registrar in Paediatrics, 2 Paediatric Neurologist, 3 Paediatrician, Teaching Hospital Karapitiya, Sri Lanka *Correspondence: sampath1188@gmail.com https//orcid.org/0000-0003-4001-512X On admission, her Glasgow Coma Scale (GCS) score was 15. Her OFC was 51cm (95 th centile) with nonbulging patent anterior fontanelle and frontal bossing.…”

“…Choroid plexus tumours are rare intracranial tumours in children and can either be papilloma or carcinoma arising from the choroid plexus epithelium 1 . These tumours can be found in isolation or very rarely with other structural malformations of the brain such as the Dandy-Walker variant 2,3 . So far, only one published case report mentioned the rare coexistence of choroid plexus papilloma (CPP) and Dandy-Walker variant 3 .…”

“…These tumours can be found in isolation or very rarely with other structural malformations of the brain such as the Dandy-Walker variant 2,3 . So far, only one published case report mentioned the rare coexistence of choroid plexus papilloma (CPP) and Dandy-Walker variant 3 .…”