Langerhans' cell histiocytosis of the head and neck in children

Abstract

This is a retrospective analysis of a 29-year institutional experience with Langerhans' cell histiocytosis (LCH) in children. Cases of LCH were categorized by disease extent into three groups: group 1, with a solitary focus of LCH; group 2, with multiple non-vital organ foci; and group 3, with vital organ disease. Sixteen patients averaging 7.3 years of age and with 11.0 years of follow-up presented in group 1. One child experienced a spontaneous remission; all other children responded to local treatment. The skull was the most common site of involvement. Eight children averaging 4.6 years in age and with 8.0 years of follow-up presented in group 2; 3 children had diabetes insipidus. Treatment included radical surgery, radiotherapy, and chemotherapy; however, disease persisted in all patients. Iatrogenic complications were a significant cause of morbidity and mortality. Only 1 patient, 1 year of age, presented with lung and liver involvement (group 3). He died after a fulminant course. Current recommendations for diagnosis, evaluation, and treatment of LCH are discussed.