Central nervous system juvenile xantogranuloma: a case report

Case presentation: Boy, 7 years old. Born at term, with no history of consanguinity or complications. Previously healthy patient. Child with a history of Attention Deficit and Hyperactivity Disorder, with adequate neuropsychomotor development. He evolved with spastic paraparesis, frequent falls, enuresis, focal epilepsy, reduced strength in the lower limbs and cutaneous plantar reflex in extension. In the investigation, neuraxial resonance showed nodular thickening of the roots of the cauda equina and the roots of the neural foramina throughout the lumbar segment, with contrast enhancement around the conus medullaris and thickening and contrast enhancement of the roots emerging from the lower thoracic segment, which may represent myelopathy or neoplasia, and nodular images located on the surface of the parietal and left frontal lobes, also increased T2/FLAIR signal in the white matter adjacent to the nodular lesions, suggesting vasogenic edema. Increased signal diffusion in the largest lesions of the right parietal lobe, with low signal on the ADC map, which may correspond to high cellularity, also suggestive of neoplasia. CSF with high protein and low glucose. Anatomopathological exam of the cerebrospinal fluid showed histiocytes and anatomopathological exam of the lesion showed xanthomatous histiocytes and lymphoplasmacytic infiltrate. Immunohistochemical profile consistent with infiltration of meninges by xanthomatous histiocytes.

Discussion: Juvenile xanthogranuloma is the most common non-Langherhans cell histiocytosis in children, mostly benign. Intracranial involvement occurs in only 2% of children and is strongly associated with leukemia. When it occurs in the nervous system, it has inexorable evolution and the treatment depends on the resectability of the lesion.

Final comments: Juvenile xanthogranuloma of the Central Nervous System is a rare neoplastic disease of severe evolution and the treatment depends on the resectability of the lesion, performed using a Langerhans cell histitiocytosis protocol, due to the aggressiveness of the condition.

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Artikel online veröffentlicht:
18. September 2023