Ear involvement in childhood Langerhans' cell histiocytosis.
BACKGROUND
Ear involvement (EI) in Langerhans' cell histiocytosis (LCH) occurs quite often. We reviewed the Italian pediatric population of 251 children with LCH diagnosed between 1982 and 1995, focusing on EI, to highlight the prevalence, the clinical presentation, the outcome during follow-up, and the prognostic impact of otologic LCH.


METHODS
EI was defined by chronic otorrhea and/or mastoid infiltration, external auditory meatus lesions, and middle/internal EI. The age at diagnosis, sex, system involved, organ dysfunction, treatment, disease control, and outcome were recorded.


RESULTS
EI was noted at presentation in 34 children (13. 5%). They had a younger age at diagnosis (p=.0013) and near totality of multisystem disease (93.8% of patients with EI). Among patients with multisystem disease, children with EI seemed to have a higher risk of poor response and a higher percentage of second line treatment (p=.003).


CONCLUSIONS
EI seems to identify patients with a particular disease behavior, which requires a more accurate evaluation at diagnosis, staging and treatment, and a strict follow-up, considering the possibility of an unfavorable outcome.
Surico
G
G
Department of Pediatric Hematology and Oncology, II Pediatric Clinic, University of Bari Piazza Giulio Cesare, 70124, Bari, Italy.
Muggeo
P
P
Muggeo
V
V
Conti
V
V
Novielli
C
C
Romano
A
A
Loiacono
G
G
Ceci
A
A
Rigillo
N
N
D000293:Adolescent; D017677:Age Distribution; D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D016001:Confidence Intervals; D018572:Disease-Free Survival; D004427:Ear Diseases / Q000453:epidemiology* / Q000209:etiology* / Q000628:therapy; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D017678:Sex Distribution; D015996:Survival Rate