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Langerhans cell histiocytosis of bone in children: a long-term retrospective study

Postini, Anna Mariaa; Andreacchio, Antoniob; Boffano, Micheleb; Pagano, Manuelaa; Brach Del Prever, Adalbertoa; Fagioli, Francaa

Journal of Pediatric Orthopaedics B: September 2012 - Volume 21 - Issue 5 - p 457–462
doi: 10.1097/BPB.0b013e32835367c0
Miscellaneous

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.

Departments of aPediatric Oncohematology

bPediatric Orthopaedics, Regina Margherita Children’s Hospital, Turin, Italy

Correspondence to Michele Boffano, MD, Department of Pediatric Orthopaedics, Regina Margherita Children’s Hospital, Piazza Polonia 94, 10126 Turin, Italy Tel: +39 338 380 9997; fax: +39 011 313 5109; e-mail: boffano@inwind.it

© 2012 Lippincott Williams & Wilkins, Inc.