[Juvenile xanthogranuloma of the central nervous system]

Juvenile xanthogranuloma (JXG) is a benign histiocytosis affecting mostly skin, although it may also be extracutaneous. Lesions tend to regress spontaneously, but there are cases of bad prognosis.

We report the case of a girl aged 3 months with JXG involving skin and, asymptomatically, central nervous system (CNS) and lungs. At age 4 months, the bigger CNS lesion, placed in the right cavernous sinus and whose growth might have had important neurologic repercussions, was excised. After surgery she presented seizures with initial response to antiepileptic drugs. At age 8 and 10 months surgery was again necessary for appearance of a tension pseudoyst in postsurgical bed; a cystoperitoneal shunt was instaured and seizures were controlled. When the patient was 12 months old, the diagnosis of hypothyroidism was established, with a favourable response to levothyroxine. At present, the patient is 2 years 8 months and has a slightly slow neurologic development, with occasional short seizures. Skin lesion is smaller, lung nodules have regressed and, regarding CNS lesions, the bigger has not recurred and the other one remains stable.

Some authors propose doing JXG s extension study only when suggested by clinical findings, so long as an effective therapy and the advantage to presymptomatic treatment are not well established. We wonder whether early diagnostic and therapeutic approach to silent deep lesions might lead to a better outcome of patients with JXG, particularly those with CNS involvement.