Case series of choroid plexus papilloma in children at uncommon locations and review of the literature

Abstract

Background:Choroid plexus papillomas (CPPs) comprise around 1% of intracranial neoplasms. The most common location is atrium of the lateral ventricle in children and fourth ventricle in adults. Other rare locations include third ventricle, cerebellopontine (CP) angle and cerebral parenchyma, with only a few cases reported. Authors report three cases of CPP at uncommon locations in pediatric patients. The rarity of these locations, diagnostic dilemma and management aspects are discussed along with an extensive review of the literature.Methods:Retrospective institutional data analysis of histopathologically confirmed pediatric CPPs from 2010 to 2014.Results:Authors noted three cases of CPP in children in uncommon locations-one each in the posterior third ventricle, fourth ventricle, and CP angle. All were males in the first decade. Two cases presented with features of obstructive hydrocephalus while the latter presented with compressive effects. Complete excision was achieved in two cases while subtotal removal was performed in one case (fourth ventricular) because of excess blood loss. Mean follow-up duration was 24.6 months (range 20–30 months). One case (of subtotal removal) had fair recovery while other two had excellent outcomes.Conclusions:Posterior third ventricle, fourth ventricle, and CP angle are uncommon locations for these tumors in children. Complete surgical removal is the treatment of choice and approach needs to be tailored according to the site and size of the lesion. Blood loss is a major concern in young children as they are highly vascular tumors. Complete removal leads to excellent long-term survival rates. Adjuvant treatment is not required.

Infratentorial choroid plexus tumors in children

“…None required blood transfusion or preoperative embolization for the posterior fossa lesions. Our radiographic findings were similar to prior studies particularly with the spiculated, calcification, contrast enhancement, and frond-like projections [ 13 , 23 ]. These frond-like tissues are relatively avascular and reduced in volume by bipolar cautery and suction.…”

Shunt ascites in a fourth ventricular choroid plexus papilloma in a young child: An uncommon case

“…In children, a few cases of fourth ventricular CPPs have been reported. [ 1 2 3 ] The most common presentation is with raised intracranial pressure as a consequence of block of cerebrospinal fluid (CSF) pathways and/or overproduction of CSF. [ 1 ] An uncommon complication following ventriculoperitoneal (VP) shunt placement in children with CPP is the development of ascites.…”

Choroid plexus papilloma of the fourth ventricle: A pediatric patient

“…[789] Just a few cases of choroid plexus papilloma of the fourth ventricle in pediatric patients have been reported, with an approximated incidence of 10%–20%. [210]…”