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.
The mother of a 1-year-old girl initially noticed reduced activity of the lower limbs which worsened over 8 days. On examination paraplegia, exaggerated deep tendon reflexes and positive Babinski sign were noted. No other neurological deficit found. Cutaneous lesions were absent. Magnetic resonance imaging (MRI) of the spine showed intradural extramedullary (IDEM) lesion at D6–D8 level suggestive of a benign nerve sheath tumor [].\nTotal excision of the tumor was done. Gross specimen included multiple bits yellowish-white, soft to firm. Histopathology showed tumor composed of sheets of spherical, ovoid, and spindle cells with vesicular bland nuclei. Many Touton giant cells were seen [].\nOn immunohistochemistry, the tumor cells were strongly positive for CD68 (histiocytic marker) and weakly for CD163 []. Cells were negative for s100 protein, CD1a, glial fibrillary acidic protein, epithelial membrane antigen. Special stain such as Zeil Neelson, Gomori methenamine silver, and periodic acid-Schiff stain were negative, thus ruling out mycobacteria and fungi as etiologic agents. Thus, a diagnosis of JXG was made. The infant showed full clinical recovery. The patient was managed with regular follow-up only and is doing well until date.