Xanthogranuloma of the choroid plexus of the third ventricle: case report and literature review

Abstract

ResumenEl xantogranuloma del plexo coroideo es un tumor benigno de etiología desconocida que raramente produce síntomas. Su comportamiento en los estudios de resonancia magnética se ha descrito en pocas ocasiones y no está bien definido. Presentamos un nuevo caso de xantogranuloma del tercer ventriculo con caraterísti-cas atípicas en los estudios de CT y RM craneal en un paciente de 47 años y una clínica de 2 meses de evolución de alteración de la marcha y deterioro cognitivo. Igualmente, se revisa la literatura existente respecto a la presentación clínica y radiológica de estos tumores.PALABRAS CLAVE: Xantogranuloma. Tumor de tercer ventrículo. RM Introduction XG are benign tumours typically composed of large foam-filled cells with clusters of lymphocytes and macrophages associated to cholesterol clefts. XG rarely cause neurological dysfunction 3,13,15 . With few exceptions, they constitute incidental autopsy findings almost always restricted to the choroid plexus of the lateral ventricles 21 . Obstruction of the ventricular system has been reported infrequently. Etiology is still debatable 17 . We report a new case of symptomatic XG of the third ventricle studied with CT and MRI and showing some particular histopathological and neuroradiological features. Case reportA 47-year-old man was admitted with a 2-month history of gait instability and urinary disturbances, consisting in occasional loss of sphincterian control and short-term memory deficits. The symptoms had developed insidiously and the patient did not suffer headache, vomiting or loss of visual acuity.Physical and routine laboratory examinations, including serum cholesterol determinations, were normal. Neurological examination revealed disturbance of recent memory and cognitive impairment. Fundoscopy was normal. He scored 24/30 on minimental test. Short stepped gait and intentional tremor were also observed.Cranial CT scan showed a 2x2x1 cm oval shaped lesion located in area of the third ventricle obstructing the foramina of Monro and causing biventricular hydrocephalus. The lesion was heterogeneous appearing partly hypodense and hyperdense, with hyperdense areas corresponding to calcification. Following contrast injection the lesion showed partial enhancement. There was associated edema in the basal ganglia of the left cerebral hemisphere ( Figure 1).Cranial MRI revealed the lesion in the region of third ventricle and hypothalamic area. It was located mainly extraventricularly, spreading between the fornices, oc- cluding both foramina of Monro and causing bilateral hydrocephalus. It was heterogeneous in appearance being isointense in pT1 sequences and hyperintense in pT2 images, showing haemosiderin deposits and calcification. Important hyperintensity extending into the left basal ganglia region suggestive of edema was also observed. Following gadolinium administration it showed partial enhancement ( Figure 2).The patient was operated through a transcallosal approach. The left foramen of Monro appeared deformated by a brown yellowish subependymal l...

Symptomatic Bilateral Xanthogranuloma of the Choroid Plexus

“…XGRs, which are sometimes also referred to as cholesterol granulomas, are most commonly found as smooth, pale-yellow masses, which are occasionally encapsulated. [ 3 ] In our present case, the lesion was quite firm, well-circumscribed, and easily separated from surrounding tissue. XGRs represent tissue reactions with a pseudotumor aspect and are composed of cholesterol clefts and inflammatory cells such as foamy cells, histiocytes, multinucleated foreign body giant cells, hemosiderin deposits, and fibrous proliferation.…”

“…The first description of an XGR was reported by Blummer in 1900. [ 3 ] In general, XGRs are asymptomatic lesions and are usually discovered incidentally. XGRs can cause obstructive hydrocephalus, and patients may develop neurological symptoms such as a headache, papilledema, gait instability, cognitive impairment, and urinary disturbances.…”

“…[ 1 ] XGR is known as cholesterol granuloma and is a rare granulomatous tumor characterized by cholesterol clefts, hemosiderin deposits, lymphocytic infiltration, and fibrous proliferation. [ 1 2 3 ] XGRs are idiopathic, and usually asymptomatic, with an incidence at autopsy of approximately 1.6%–7.0%. [ 2 ] The prevalence of XGRs appears to peak at 20–40 years of age.…”

“…[ 2 ] The prevalence of XGRs appears to peak at 20–40 years of age. [ 1 2 3 ] The majorities of symptomatic cases concern the choroid plexus of the third ventricle and predominantly lead to obstructive hydrocephalus. [ 3 ]…”

A “pseudo-cavernoma” – An encapsulated hematoma of the choroid plexus

“…Xanthogranulomatous reaction may involve the choroid plexus bilaterally, but this typically reveals “foamy” cells, or lipid-laden macrophages and multinucleated giant cells, which our specimen lacked. (9)…”