Xanthogranuloma of the cervical spine

Case contributed by Maksim Malakhov
Diagnosis certain

Presentation

Weakness of upper limbs and reduced activity. Due to long-standing fever of unknown etiology, his CSF was examined (inflammatory markers revealed). MRI of head and spine was performed.

Patient Data

Age: 5 months
Gender: Male

Dorsally located ovoid-shaped intradural extramedullary lesion at the level of C1-C5 vertebral bodies. The lesion was isointense (or slightly hyperintense) to the spinal cord on T1WI, slightly hypointense on T2WI, enhanced homogeneously on contrast, and likely dural-based.

Computed tomography showed no bone destruction

Histologic examination

pathology

Histologic examination showed dense lymphohistiocytic proliferation with foamy Touton multinucleated giant cells (arrows). The histologic conclusion was extracutaneous juvenile xanthogranuloma.

Case Discussion

The tumor was removed surgically. Microscopic (histologic) conclusion is presented above.

Solitary cervical spine xanthogranuloma is an extremely rare tumor2,3 and as yet, specific MRI features have not been described in the literature. The radiological presentation is variable1. It could be infiltrative, invasive, with multiple nodular lesions along nerve roots, and with bony destruction. Due to its rarity and variabile appearance on MRI, the inclusion of juvenile xanthogranuloma in the differential list of intradural-extramedullary lesions could be problematic; other more common lesion, such as meningioma, are more expected.

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