Si. Angeli et al., LANGERHANS CELL HISTIOCYTOSIS OF THE HEAD AND NECK IN CHILDREN, The Annals of otology, rhinology & laryngology, 104(3), 1995, pp. 173-180
This is a retrospective analysis of a 29-year institutional experience
with Langerhans' cell histiocytosis (LCH) in children. Cases of LCH w
ere categorized by disease extent into three groups: group 1, with a s
olitary focus of LCH; group 2, with multiple non-vital organ foci; and
group 3, with vital organ disease. Sixteen patients averaging 7.3 yea
rs of age and with 11.0 years of follow-up presented in group 1. One c
hild 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 foll
ow-up presented in group 2; 3 children had diabetes insipidus. Treatme
nt included radical surgery, radiotherapy, and chemotherapy; however,
disease persisted in all patients, Iatrogenic complications were a sig
nificant cause of morbidity and mortality. Only 1 patient, 1 year of a
ge, 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.