Symptomatic Bilateral Xanthogranuloma of the Choroid Plexus

Abstract

Xanthogranulomas (XGRs) of the choroid plexus are rare, asymptomatic, and benign lesions usually found incidentally. Here, we present a case of a 47-year-old male with bilateral XGR of the choroid plexus with periventricular edema and discuss our case in relation to a review of existing literature pertaining to the radiology of XGRs. To the best of our knowledge, this is the first reported case of bilateral trigonal XGR causing brain edema without ventricular dilatation. Despite the fact that they can cause hydrocephalus, XGRs are silent and benign lesions. Although the etiopathology of XGRs remains poorly understood, enhanced imaging analyses may provide additional information regarding edema and focal white matter signal changes.

Case Presentation: Unusual Association Between Possible Bilateral Intraventricular Xanthogranulomas, Postero-inferior Cerebellar Artery Aneurysm and Thrombophilia

“…XG usually remains asymptomatic, because the size of the masses is too small to obstruct the cerebrospinal fluid (CSF) flow. They become symptomatic when they have increased in size enough in order to be clinically significant by obstructing CSF flow, the most frequent symptomatic XG being located in the third ventricle where they obstruct the foramen of Monro resulting in hydrocephalus [15].…”

“…Regarding frequency and significance, the symptomatology encountered was: headache, nausea and vomiting, seizures, ataxia, nystagmus, double vision, hemiparesis, bladder incontinence, deterioration of consciousness, changes of personality and other symptoms secondary to intracranial hypertension [15,18]. In our case, the patient had a progressive symptomatology over the course of several years, which could not be explained by other associated pathologies, as there was no improvement after the arachnoid cyst was removed and the posterior spinal cord atrophy could explain the paresthesias, but not the limb weakness.…”

Bilateral xanthogranuloma of the choroid plexus in an infant

“…Xanthogranulomas of the choroid plexus are benign and rare lesions occurring in the choroid plexus of the lateral and third ventricles. [ 1 2 ] Though on occasion, they may cause hydrocephalus or intraventricular hemorrhage, they are usually asymptomatic and found incidentally while imaging for other conditions. [ 1 2 ] They are usually bilateral[ 3 ] and have an autopsy incidence of 1.6%–7.0%[ 1 2 ] with many of these being extremely small and insignificant.…”

“…[ 1 2 ] Though on occasion, they may cause hydrocephalus or intraventricular hemorrhage, they are usually asymptomatic and found incidentally while imaging for other conditions. [ 1 2 ] They are usually bilateral[ 3 ] and have an autopsy incidence of 1.6%–7.0%[ 1 2 ] with many of these being extremely small and insignificant. They are different from choroid plexus cysts which are commonly seen on antenatal ultrasound (1% of all cases) and are formed by infolding of the neuroepithelium into the stroma and usually disappear by the 28th week of gestation.…”

“…Yetkinel and Bek[ 2 ] have described their micro-architecture as consisting of “cholesterol clefts, lymphocyctic infiltration, giant cells with multiple nucleoli, foamy macrophages (xanthoma cells), fibrous proliferation, and hemosiderin deposits.” Several causes of pathogenesis ranging from cellular degeneration (desquamation of cuboidal epithelial cells and their sequestration), tissue reaction to hemorrhage, and even systemic disturbances of lipid metabolism have been put forth. [ 1 2 ] Hemorrhages at the periphery of these lesions or pathological accumulation of lipids leading to inflammation may manifest as hyperintensities adjacent to the lesions on T2-weighted sequences. In our patient, hyperintensities in the white matter might also have been consequent to the hemorrhage in the cysts or a sequelae of meningitis.…”