Intradural Juvenile Xanthogranuloma with Involvement of Multiple Nerve Roots. A Case Report and Review of the Literature.

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Intradural Juvenile Xanthogranuloma with Involvement of Multiple Nerve Roots. A Case Report and Review of the Literature.

World Neurosurg. 2018 Aug 07;:

Authors: Wolfe C, El Ahmadieh TY, Aoun SG, Vance AZ, Hatanpaa KJ, Wohlfeld B

BACKGROUND: Juvenile Xanthogranuloma is a rare, non-Langerhan’s cell histiocytic disorder that primarily presents as multiple cutaneous lesions in young males. Solitary lesions in the spinal column are a particularly rare presentation of this disease, and CNS involvement can portend a poor prognosis. This report highlights an unusual case of an adult woman with an unresectable JXG of the lumbar spine. A review of the published cases of thoracolumbar JXG and the current state of the literature in regard to diagnosis and treatment is presented.
CASE DESCRIPTION: A 28-year-old woman presented with back pain and worsening lower extremity pain, numbness and weakness. MRI demonstrated an enhancing lumbar mass, but at the time of surgery, no discrete mass was identified. Multiple roots were grossly enlarged, and electrical stimulation identified the L4 root as the most abnormal. Despite an attempt to debulk, the majority of the mass could not be safely removed. The patient had incomplete improvement of symptoms post-operatively but elected to forgo chemotherapy. Three-month follow-up imaging showed active lumbar spinal disease, and imaging and follow up at 27-months has revealed no changes. Her symptoms have been satisfactorily controlled with conservative therapy.
CONCLUSIONS: Juvenile Xanthogranuloma of the spine is a rare disease who’s nonspecific clinical and radiographic findings can make difficult to diagnose and dictates the use of immunohistochemical staining. If possible, total surgical resection offers the greatest outcomes, but other modalities such as chemotherapy may be viable alternatives or adjuvants.

PMID: 30096507 [PubMed – as supplied by publisher]

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