title: Langerhans cell histiocytosis of bone in children:  a long-term retrospective study.
abstract: Langerhans cell histiocytosis is rare and frequently involves the bone. We retrospectively reviewed the orthopaedic aspects (symptoms, localizations, treatments) and the long-term outcome [disease status, overall survival (OS), event-free survival (EFS)] of 121 patients (June 1968-December 2009). The main symptom was local pain. The orthopaedic treatment was mainly conservative. The most frequent localization was osseous monofocal (62% of monosystemic diseases). Monosystemic and osseous monofocal localizations, treatment after 1991 (OS, P=0.007; EFS, P=0.03) and age older than 2 years (OS, P=0.003; EFS, P=0.001) were prognostic factors that were positively associated with survival. Oncologic treatment has improved over time, translating into better survival. A biopsy is often mandatory.
authors.affiliation: Department of Pediatric Oncohematology, Regina Margherita Children's Hospital, Turin, Italy.
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Postini
authors.forename: Antonio
authors.initials: A
authors.lastname: Andreacchio
authors.forename: Michele
authors.initials: M
authors.lastname: Boffano
authors.forename: Manuela
authors.initials: M
authors.lastname: Pagano
authors.forename: Adalberto
authors.initials: A
authors.lastname: Brach Del Prever
authors.forename: Franca
authors.initials: F
authors.lastname: Fagioli
mesh_terms: D000293:Adolescent; D001847:Bone Diseases / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007558:Italy / Q000453:epidemiology; D008297:Male; D010146:Pain; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome