LANGERHANS CELL HISTIOCYTOSIS - PRESENTATION AND EVOLUTION OF RADIOLOGIC FINDINGS WITH CLINICAL CORRELATION

Radiologic images and medical records of 42 children with Langerhans c ell histiocytosis (LCH) (Histiocytosis X) were reviewed to evaluate th e presentation of the disease and the evolution of the radiologic find ings, There were 26 male and IG female patients aged 3 months to 18 ye ars. Twenty-two patients presented with localized disease; 20 presente d with multifocal disease, Four patients developed diabetes insipidus, Two patients had organ dysfunction. The radiologic findings were larg ely due to destructive bone lesions; 83% of the patients had at least one affected bone, Isolated soft-tissue masses, interstitial lung dise ase, and central nervous system abnormalities were also seen, Of patie nts in whom results of appropriate follow-up were available, 91% showe d improvement in their lesions, 43% developed new lesions, and 92% had good clinical outcomes, LCH is usually a self-limited disease with a varied clinical and radiologic presentation. The prognosis is generall y poor in children with organ dysfunction. In the absence of organ dys function, children with either localized or multifocal LCH have an exc ellent prognosis.