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

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 pres...

Full description

Saved in:
Bibliographic Details
Published inAsian journal of neurosurgery Vol. 9; no. 4; p. 239
Main Authors Purohit, Devendra, Chanduka, Amit, Sharma, Vinod, Mittal, Radhey, Singhvi, Shashi
Format Journal Article
LanguageEnglish
Published A-12, 2nd Floor, Sector 2, Noida-201301 UP, India Thieme Medical and Scientific Publishers Pvt. Ltd 01.10.2014
Medknow Publications and Media Pvt. Ltd
Medknow Publications & Media Pvt Ltd
Subjects
Care and treatment
CASE REPORT
Case studies
Diagnosis
Nervous system cancer
Patient outcomes
Risk factors
Xanthoma
India
Adult
compressive myelopathy
epidural
juvenile xanthogranuloma
langerhanscell histiocytosis
thoracic spine
Online AccessGet full text

Cover

More Information
Summary: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.
ISSN:1793-5482
2248-9614
DOI:10.4103/1793-5482.146640