Multiple Cerebral Juvenile Xanthogranuloma; a Case Report

Juvenile xanthogranuloma (JXG), is the most common form of non-Langerhans cell histiocytosis. It is a rare and usually benign, monoclonal proliferative disorder of histiocytic cells. The occurrence of multiple brain lesions is extremely rare. Due to the rarity of CNS disease, the preferred treatment and overall prognosis of these patients remain unclear. In this case report, authors present their experience about a patient with isolated multiple cerebral JXG which yields considerable outcomes. The patient was an eleven-year-old boy with anorexia and recurrent vomiting. Brain MRI demonstrated multiple lesions involving right lateral ventricle wall and periventricular white matter, the right side of splenium, mammillary body and tuber cinereum, fourth ventricle floor and roof, and cerebellar tonsil. After initial response to chemotherapy, the lesions recurred and radiotherapy was done. Consequent to good response after radiotherapy and despite the occurrence of a seizure attack, long term follow-up showed the marked resolution of the lesions and a good general status.