A 50-year-old male patient was referred to our hospital, with a 4-month history of neck pain and gradually progressive weakness of all four limbs. Over the past 2 weeks, the patient noticed significant deterioration and became bed bound. The patient denies any previous history of trauma, malignancy, or medical history of note. Clinical examination revealed spastic quadriparesis of 3/5 with 80% decreased sensation of all modalities below C5. Sphincters were found to be intact.\nPlain X-ray cervical spine on anterior-posterior (AP) view showed a large lucent lesion replacing the C5 vertebra with loss of the left pedicle []. The magnetic resonance imaging (MRI) of the cervical spine demonstrated a T1 and T2 isointense, expansile mass lesion involving the C5 vertebral body extending into the left pedicle but sparing neural foramina. On T2, small hyperintense intralesional cavity was noted in the anterior part. The lesion extended across the C5/6 intervertebral disc and into the anteroinferior part of the C4 vertebra. Significant cord compression with absent thecal sac against C5 was noted. The lesion enhanced homogeneously with gadolinium and appeared completely extradural [Figure -]. Based on imaging, metastasis, myeloma, and tuberculous granuloma were suspected.\nThe patient underwent standard anterior cervical decompression and fixation. Peroperatively, tumor appeared as a well-encapsulated, firm, multinodular mass, yellow tan in color, 3 cm × 3 cm × 2 cm in size. There was a small necrotic part corresponding to the T2 hyperintensity. The lesion was excised in piecemeal fashion [] to completely decompress the cord. No clue of bone was found within the mass. At this point, differential was further narrowed to granulomatous lesion like tuberculosis. Histology revealed sheets of histiocytes with foamy cytoplasm and bland nuclei. Focal cholesterol clefts and multinucleated giant cells were also noticed. There was no cellular atypia. CD68 was positive and S100 was found to be negative [Figure and ].\nThe serum calcium was normal, and lipid profile showed normolipemia. Clinical examination did not reveal any soft-tissue xanthomas. The patient was able to return to his normal activities 4 weeks after the operation. Postoperative imaging as per routine following anterior decompression and fixation consisted of plain X-ray cervical spine AP and lateral views [Figure and ]. Postoperative computed tomography (CT) of the same area was planned at the 6-month follow-up both to see the integrity of fixation as well as to rule out the tumor recurrence, but the patient was not able to reach out for imaging because of pandemic restrictions. Currently, he is 1 year out of his surgery, with no reported complaint as per telephonic follow-up.
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.