Rare presentation of juvenile xanthogranuloma in the thoracic spine of an adult patient: case report and literature review

Abstract

Isolated spinal involvement of juvenile xanthogranuloma (JXG) is extremely rare. There are only seven prior published cases of spinal JXG, of which only one has been reported in an adult. We report here the eighth case of spinal JXG and the second in an adult. The patient, a 22-year-old female, presented with progressive upper backache. Radiological examination revealed a well-defined osteolytic hypointense mass in the T7 vertebral body, with a large soft tissue paraspinal extension causing cord compression. Complete resection of the mass was performed, with resolution of symptoms. Histology showed a histiocytic tumour with numerous Touton, foreign body and osteoclastic giant cells, immunopositive for CD68 and vimentin and negative for S100 and CD1a, corresponding to a diagnosis of JXG. Literature regarding spinal JXG is reviewed and discussed.

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

“…Search for spinal involvement yielded only nine cases of spinal JXG [ Table 1 ]. [ 2 4 6 7 8 9 10 11 12 ] Here, we report the tenth in the series and fifth case to be reported in adult spine. As reported, spine can be involved at any location but an overall predilection for thoracic spine can easily be noticed.…”

Solitary juvenile xanthogranuloma in cervical spine: case report and review of literature

“…Cao et al described a patient who had JXG in the upper cervical spine, along with bony destruction, necessitating bony fusion following excision of the tumor (3). Jain et al reported a patient with D7 bony lesion with paraspinal and anterior epidural components, mimicking an aneurysmal bone cyst (12). Intraoperatively, these tumors mimic IDEM tumors like schwannomma or meningiomas, or bony tumors like aneurysmal bone cysts, depending on their radiological appearance.…”

“…In case of presentation as IDEM tumors, resection of the tumor appears to be curative (14,19,20). However, fusion may be required following radical excision for lesions having extensive bony involvement (3,12). Immunohistochemistry shows the lesions to be positive for factor XIIIa, CD68, CD163, fascin, and CD14 but are negative for S100 and CD1a (12,16,17).…”

“…However, fusion may be required following radical excision for lesions having extensive bony involvement (3,12). Immunohistochemistry shows the lesions to be positive for factor XIIIa, CD68, CD163, fascin, and CD14 but are negative for S100 and CD1a (12,16,17). It is important to distinguish these tumors from LCH, as these lesions are more aggressive, and require aggressive treatment.…”

Juvenile Xanthogranuloma–A Rare Cause of Unilateral Orbital Swelling in an Elderly Patient

“…One such case discusses a rare presentation of JXG found in the thoracic spine. 6 Complete resection of the mass was undertaken with resolution of symptoms. 6 Histological exam showed numerous Touton cells confirming the diagnosis of non-Langerhan's histiocytic disorder JXG providing evidence to the importance of histopathological exam.…”