Isolated cervical juvenile xanthogranuloma in childhood

Study Design. This is a report of an exceptional case of isolated cervical juvenile xanthogranuloma in a child. Objectives. This case report draws attention to the fact that isolated xanthogranuloma of the central nervous system should be considered among possible diagnosis of subdural extramedullary spinal masses in children and young adults. Summary and Background Data. Isolated juvenile xanthogranuloma of the central nervous system is extremely rare. When located in the spinal canal it behaves like any extramedullary mass-occupying lesion. MRI depicts the tumor's association with adjacent structures. In cases in which a subtotal surgical removal was possible, radiotherapy has been indicated. Methods. A three-year-old girl presented severe pain in the right shoulder and spastic tetraparesis. The MRI showed an intradural extramedullary mass homogeneously enhancing after DTPA-gadolinium infusion. Complete surgical removal of the tumor was performed through open-door laminoplasty. Results. The child was pain free immediately after the surgical removal of the tumor. A gradual complete recovery of the neurologic deficits followed. Open-door laminoplasty provided sufficient operative space, and it minimized the impact on the growing spinal column. Conclusions. Isolated juvenile xanthogranuloma does not show any predilections of localization inside the central nervous system. Both intracranial and spinal juvenile xanthogranulomas appear isointense in MRI and enhance homogeneously with gadolinium. Whenever possible, total surgical removal alone seems to be curative. Otherwise, a subtotal removal of the tumor might be followed by radiotherapy. Immunohistochemical tests ensure the diagnosis.