“An 18-year-old male presented with a short history of 8 days of weakness of both lower limbs. There was no history of trauma or tuberculosis or skin lesions in form of papules or macules. No other complaint was associated and the past history was of no significance. The cranial neurological examination was normal. His Bulk, tone, and power in both upper limbs were normal. On examination of both lower limb, his bulk was normal, had bilateral spasticity with muscle power of grade 4 in all groups of muscles. His deep tendon reflexes were exaggerated with bilateral extensor plantar. No sphincter involvement and all routine investigations including lipid profile were normal. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement atD7 to D10 vertebral segment. Mass lesion was isointense on T1 and hyper-intense on T2 with no contrast enhancement [Figure , ].\nD7 to D10 Laminectomy with complete excision of firm epidural mass, which could be separated from dura with difficulty, was carried out. Histopathology revealed fibro-collagenous tissue with dense infiltration of lymphocytes, histiocytes, plasma cells, eosinophils, and multinucleated Touton giant cells. Histiocytic granulomas were seen. Presence of arteritis and panniculitis noted. Overall morphology was suggestive of JXG. Immune marker study was proposed and revealed Viamentin positive, Cluster Differentiation-68 positive in Touton giant cell and negative for S-100, CD1a, and Actin smooth muscle [Figure –].\nPost-operative, patient improved neurologically. His power improved to grade 5/5 with decreased spasticity. At 9 month follow-up patient had mild spasticity with extensor plantar. His MRI carried out at 3 months shows post-Laminectomy status with no residual tumor [].”
A 15-year-old male presented with pain in the nape of the neck and gradually progressive ascending weakness of all four limbs along with bladder and bowel involvement for 3 years. On examination, tone was increased in all four limbs with 4/5 power in bilateral upper limbs and 2/5 power in both lower limbs, along with exaggerated deep tendon reflexes in all four limbs. Superficial abdominal reflexes were absent and, bilateral planters were extensor. He had 70–80% sensory loss below T4 level. His routine laboratory studies including hematological and biochemical parameters were within normal limits.\nComputerised tomography scan of the cervical spine revealed a large osseo-destructive lesion involving body of C5and C6 vertebra which was causing spinal canal compromise [Figure and ]. The lesion was involving surrounding structures with extension into the submuscular plane anteriorly. On magnetic resonance imaging (MRI) evaluation, the mass was isointense on T1 and T2 weighted sequences with minimal contrast enhancement. It was causing distrucation of C5 and C6 vertebra body with compression of thecal sac [Figure –].\nUsing anterior cervical approach, the tumor resection was performed. Intra-operatively, the tumor involved whole of the C5 and C6 vertebral body. There was a soft tissue component of the tumor, which was extending into the submuscular plane. Inside the capsule, the tumor consisted of cheesy material that was minimally vascular. Total tumor excision was done with tricorticate iliac crest bone grafting and plate and screw fixation between C4 and C7 vertebral bodies.\nMicroscopic examination showed spindle cells disposed of in intersecting fascicles intermingled with mononuclear xanthomatous cells displaying round nuclei with dense chromatin inconspicuous nucleoli and pale eosinophilic finely vacuolated chromatin. Spindle cells display bland appearing nuclear chromatin and eosinophilic cytoplasm. Occasional cells displayed nuclear atypia and enlargement. Scattered neutrophils were seen admixed with these cells. Focal areas of calcification and occasional osteoclasts like giant cells were also seen. Interspersed areas showed degenerated bony trabeculae and cartilage [Figure and ].\nPostoperatively, patient had significant improvement in neck pain and spasticity in all four limbs. At follow-up of 6 months, he was doing well.