Solitary juvenile xanthogranuloma in cervical spine: Case report and review of the literature

摘要

Solitary juvenile xanthogranuloma (JXG) in the spinal column is extremely rare. Here, we report and characterize the case of xanthogranuloma of the upper cervical spine. A 18-year-old male presented with neck pain for 3 months, along with progressive quadriparesis and sensory loss of 2 months duration with urinary retention. Motor examination revealed spastic quadriparesis with power of 2/5 in all the 4 limbs. Magnetic Resonance Imaging (MRI) spine with contrast showed a dorsally placed intradural extramedullary lesion at the level of C2-C4 vertebral body. The lesion, measuring 2.9x1.7x1.4 cm, was isointense on T1WI, hypointense on T2WI, and enhanced homogenously on contrast. He underwent an emergency C2-C4 laminectomy and complete excision of the lesion. At 3-month follow-up, he was asymptomatic except for mild neck pain. MRI scan of the cervical spine done at follow-up, revealed complete excision of tumor without any residual lesion. Histopathological examination of the mass revealed a polymorphous population of sheets of bloated pale foamy histiocytes (xanthoma cells), numerous admixed mature lymphocytes and several Touton giant cells. The cells were positive for CD68, a histiocytic marker, and negative for CD1a (excludes LCH) and S-100 (excludes RDD).