Atypical Choroid Plexus Papilloma of the Fourth Ventricle in an Adult: A Case Report

Abstract

Atypical choroid plexus papilloma (aCPP) is very rarely seen in adults. Here, we present the case of a 47-year-old male with several months of headache, nausea, dizziness, and imbalance who was found to have an enhancing mass of the fourth ventricle with imaging findings suggestive of likely ependymoma. The patient underwent suboccipital craniotomy with C1 laminectomy and telovelar approach for gross-total resection of the lesion, with final pathology demonstrating WHO grade II aCPP. Subsequent genomic analysis showed a biologically relevant TERT mutation, as well as several variants of unknown significance. We conclude that aCPP is a rare, benign entity diagnosed by tissue sample that is potentially curative with surgical resection and may harbor targetable genetic mutations.

Foramen of Monro choroid plexus papilloma: An extremely rare location managed by endoscopic resection

“…Choroid plexus papilloma comprises less than 1% of brain tumors and even 0.5% percent of brain tumors in adults [1 , 3] . It is more common in males and can present at any age, with a higher predilection towards children [1 , 2] .…”

“…CPP can be found incidentally or present with various neurological symptoms such as headache, gaze, and vision problems, or a decrease in mental status, based on the location and size of the mass [2 , 3] . Moreover, symptoms of raised intracranial pressure and hydrocephalus remain frequently encountered, which is similar to the refractory headache and recent vomiting episodes in our patient [2 , 3] . Of notice, the hydrocephalus can develop either due to obstruction of the CSF flow or due to overproduction of the CSF from the tumor [1] .…”

“…In addition, ultrasonographic modalities can be considered in younger patients if the fontanelles are open and as fast, safe, and non-invasive modalities to assess and exclude bleeding or vascular injuries [2 , 8] . Nevertheless, tumor biopsy and histopathological examination remain the “gold standard" and definitive diagnosis cannot be established without a biopsy as it diagnoses and differentiates between CPT subtypes [1] , [2] , [3] . Spinal MRI can be considered to evaluate for CSF seeding as cases were reported with both CPP and CPC, although much more common with CPC [9] .…”

“…The symptoms of increased intracranial pressure and hydrocephalus remain the most frequent presentation in most cases [1] . However, other neurological symptoms and focal neurological deficits can be the first manifestation, or they can be found incidentally [2 , 3] . The diagnosis of such tumors is done through imaging modalities such as computerized tomography (CT) and magnetic resonance imaging (MRI), although histopathological examination remains the cornerstone of definitive diagnosis, as imaging techniques are unable to differentiate between the different types of choroid plexus tumors or other types of brain tumors [4 , 5] .…”

Rehabilitation of lateral gaze palsy in a patient with ‘Choroid Plexus Papilloma’

“…Manual Muscle Test (MMT) plexus papilloma (CPP) is an uncommon, benign carcinoma of the CNS that develops through the lining of the ventricles' choroid plexus(Wendt et al, 2022). CPP are infrequent interventricular and neuroectodermal tumours(Mazur-Hart et al, 2022).Only about 1% of adult cerebral tumours are caused by it(Goel et al, 2022) and 3-4 % in paediatric(Wendt et al, 2022). These tumours most frequently develop in the fourth ventricle in adulthood but frequently discovered in the lateral ventricles of youngsters(Pfeifer et al, 2020).…”