A girl with a stiff neck

Abstract

Juvenile xanthogranuloma (JXG) is a rare form of non-Langherans cell histiocytosis (non-LCH) observed almost exclusively in infants and young children. It is rarely systemic, involving extracutaneous sites, such as the liver, lungs, spleen, kidney, pancreas, bone or central nervous system. Systemic JXG may be associated with significant complications requiring aggressive medical or surgery care; especially, central nervous system lesions are difficult to treat and reported to be possibly fatal. Clinical presentation of JXG of central nervous system is not specific and is related to the involved site while magnetic resonance imaging examination remains the first choice for localizing the lesions. If no other system is involved, surgical excision could be sufficient.