Juvenile Xanthogranuloma of adult spine: A rare case and review of literature

Abstract

Juvenile Xanthogranuloma (JXG) is a rare disorder of central nervous system. It rarely produces compressive myelopathy. On reviewing world literature, we could find only nine cases of this disease involving spine and of which only four cases were in adults' i.e., 18 years and above. We are presenting a case of Spinal JXG in an18-year-old male with thoracic compressive myelopathy presenting as short duration progressive paraparesis. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement at D7 to D10 vertebral segment. It was isointense on T1 and hyperintense on T2 with no contrast enhancement. D7 to D10 Laminectomy with complete excision of firm epidural mass was carried out. The histopathology with tumor markers confirmed the diagnosis of JXG. Post-operative neurological recovery in this patient was good. His power improved to grade 5/5 with decreased spasticity. Follow-up MRI at 3 months showed no residual tumor. This case appears to be the first in the series with entirely extradural component in adult thoracic spine.

Case of compressive myelopathy due to juvenile xanthogranuloma of cervicothoracic junction in a 28-year-old male

“…We combined our case with the 12 other cases of spinal JXG found in the literature [ Table 1 ]. These 13 cases of JXG included six males[ 5 , 9 , 10 , 12 , 13 ] and seven females[ 1 , 2 , 6 - 8 , 11 , 15 ], typically in their late 20s to 30s [ Table 2 ]. [ 3 ] Most were located in the cervical spine, with only our patient having a spinal tumor at the cervicothoracic junction [ Table 2 ].…”

“…He was discharged a few days later, was fully neurologically intact within 2 postoperative months, and continues to be followed with repeat MRI studies (i.e., every 6-12 months) that have remained negative for tumor recurrence. Purohit et al 2014 [13] 18 M Lower limbs weakness 8 days T7-T10 extradural Laminectomy T7-T10 Total Mild spasticity (9 month) Pirillo et al 2017 [12]…”

“…These lesions may produce neurological deficits reflecting their anatomic spinal locations. [ 13 ] Here, a 28-year-old male presented with the 1-year onset of a progressive quadriparesis with 3 months’ of precipitous worsening attributed to a C6–T1 JXG that was successfully managed with a decompressive laminectomy for gross total surgical resection and C6–T2 fusion.…”

“…M 1]. ese 13 cases of JXG included six males [5,9,10,12,13] and seven females [1,2,[6][7][8]11,15] , typically in their late 20s to 30s [Table 2]. [3] Most were located in the cervical spine, with only our patient having a spinal tumor at the cervicothoracic junction [ 1].…”