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. . 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 lesion adhered to the body of the left fornix. Total excision preserving both fornices was achieved. Histopathological examination revealed a lesion composed of foamy cells in relation to vascular lines corresponding to choroids plexus showing changes secondary to haemorrhage and fibrosis which would explain the absence of the typical epithelial covering of the choroid plexus. The histological diagnosis was xanthogranuloma ( Figure 3).Postoperative MRI control showed disappearance of perilesional edema and mass ef...

Unusual presentation of adult xanthogranuloma

“…(8) It is poorly understood whether adult forms are also associated with extracutaneous involvement, with only anecdotal reports in the current literature to suggest that the breast, (9) eye (10) and central nervous system (11) could be affected. In the aforementioned cases of suggested extracutaneous involvement, the patients presented with breast lumps, nontraumatic iritis and hyphaema, and gait disturbances, respectively.…”

Adult onset xanthogranuloma – case report and review of literature

“…While no case of adult onset xanthogranuloma with concomitant cutaneous and extracutaneous lesions has been reported, cases of solitary extracutaneous xanthogranulomas located in the breast (33), eye (34), and central nervous system (35) without coexisting skin lesions have been described.…”

Xantogranuloma sintomático del ventrículo lateral: A propósito de un caso

“…La patogénesis del xantogranuloma es controvertida. Ciertos autores (Bruck et al 2 y Miranda et al 5 ) sostienen como mecanismo patogénico los cambios inflamatorios debido a la invaginación y proliferación del plexo coroideo o la presencia de hemorragias intralesionales. El plexo coroideo está formado por ejes fibrovasculares revestidos por una capa de células epiteliales cúbicas, que con el envejecimiento tienden a la estratificación y a presentar metaplasia escamosa.…”

“…Radiológicamente, los xantogranulomas se han descrito como lesiones localizadas en los ventrículos laterales, hipodensas en los estudios de TC basal (por el contenido graso), con calcificaciones estromales, similares a las calcificaciones fisiológicas del plexo coroideo, y sin realce tras la administración de contraste 5 . Sin embargo, en la literatura se han comunicado apariencias diversas con lesiones iso o hiperdensas 1 respecto al parénquima y presencia de realce parcial o intenso 1,4 .…”

“…Generalmente son hallazgos incidentales de autopsia (1,6 -7% de las autopsias) 5 , ya que la mayoría son asintomáticos 1 . Presentamos un caso de xantogranuloma de ventrículo lateral sintomático con hallazgos particulares en TC y RM.…”