Rapidly growing, multifocal, benign choroid plexus tumor in an infant: case report

Abstract

<jats:p>Choroid plexus papillomas (CPPs) are rare, benign tumors that can arise in young children. Most pediatric patients present with signs of hydrocephalus and require immediate treatment. The natural history of choroid plexus tumors in children without hydrocephalus is poorly defined. In this report, the authors present the very rare case of a child without hydrocephalus but with two intraventricular choroid plexus tumors discovered shortly after birth. Initial imaging had been performed for seizures and showed agenesis of the corpus callosum and enhancing tumors in the third and left lateral ventricles. Sequential imaging demonstrated rapid growth of both tumors. The lateral tumor was removed when the child was 3 months of age. A histological examination of the specimen showed benign features with an elevated mitotic rate. Given the patient’s age of under 3 years, the diagnosis was WHO grade I CPP. The third ventricle tumor grew rapidly. A second surgery was performed and this tumor was resected. Again, the pathological diagnosis was WHO grade I CPP. The authors present this rare case and discuss the current relevant literature.</jats:p>

Embolization of a third ventricular choroid plexus papilloma: illustrative case

“… 8 These tumors are derived from choroid plexus epithelium and present as CPP, aCPP, and CPC. 2 Choroid plexus tumors have been shown to cause hydrocephalus by both obstructing normal cerebrospinal fluid pathways and overproducing cerebrospinal fluid. 9 Gross total resection (GTR) is the treatment of choice and usually has good outcome for choroid plexus tumors, although CPCs are more aggressive and have a higher rate of recurrence.…”

“… 1 These tumors are pathologically divided into World Health Organization (WHO) grade I choroid plexus papilloma (CPP), WHO grade II atypical CPP (aCPP), and WHO grade III choroid plexus carcinomas (CPCs). 2 These tumors are typically found in the lateral ventricle in children and more commonly in the fourth ventricle in adults. Choroid plexus tumors are much less frequently found in the third ventricle and have been reported in several case reports in the literature.…”

Microscopic and endoscopic “chopstick” technique removal of choroid plexus papilloma in the third ventricle of an infant: a case report with systematic review of literature

“…Ultrasound examination performed at other hospitals suggested hydrocephalus. Cranial magnetic resonance imaging (MRI) showed the presence of a space-occupying lesion in the third ventricle, about 3.8×4.6×3.1 cm ( 3 ) in size, isosignal at T1 and mild high signal at T2-weighted images, accompanied with severe hydrocephalus ( Figures 1A, B ). Contrast-enhanced lesions showed “cauliflower-like” appearance on MRI ( Figures 1C, D ).…”

“…CPP is most often located in the lateral ventricles, with few reported cases in the third and fourth ventricles ( 2 ). Typical clinical manifestations of CPP include hydrocephalus, macrocephaly, and growth retardation ( 3 , 4 ). The most common treatment for CPP is surgery.…”

Infantile epileptic spasms syndrome as an initial presentation in infantile choroid plexus papilloma: A case report

“…CPP is commonly found in the lateral ventricles with a rate of 43%–65%, followed by the fourth and the third ventricle, and less in other regions ( 3 , 4 ). Hydrocephalus, macrocephaly, enlarged fontanelles, and symptoms of intracranial hypertension are noted in nearly all clinical cases with CPP ( 5 , 6 ). However, infantile epileptic spasms syndrome as the initial clinical finding in such patients is rare, and only a few cases have been reported in the literature ( 7 ).…”