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Langerhans cell histiocytosis of bone in children: a long-term retrospective study
Anna Maria Postini1, Antonio Andreacchio, Michele Boffano
1Department of Pediatric Oncohematology, Regina Margherita Children's Hospital, Turin, Italy.
Insights
Langerhans cell histiocytosis, a rare bone disease, shows improved survival with modern oncologic treatments. Early diagnosis and conservative orthopedic management are key for better outcomes in pediatric patients.
Area of Science:
- Orthopedic Surgery
- Pediatric Oncology
- Histiocytosis Research
Background:
- Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease.
- Bone involvement is common in LCH, presenting orthopedic challenges.
- Long-term outcomes and prognostic factors require further elucidation.
Purpose of the Study:
- To retrospectively review orthopedic aspects of LCH.
- To evaluate long-term disease status, overall survival (OS), and event-free survival (EFS).
- To identify prognostic factors influencing patient survival.
Main Methods:
- Retrospective review of 121 LCH patients (June 1968-December 2009).
- Analysis of orthopedic symptoms, bone localizations, and treatments.
- Assessment of long-term outcomes including disease status, OS, and EFS.
Main Results:
- Local pain was the primary symptom; conservative orthopedic treatment was most common.
- Osseous monofocal localization was frequent in monosystemic LCH.
- Positive prognostic factors for survival included monosystemic/osseous monofocal disease, treatment post-1991, and age >2 years.
Conclusions:
- Improved oncologic treatment over time has enhanced survival rates for LCH patients.
- Monosystemic and osseous monofocal LCH, along with modern treatment protocols, are associated with better prognoses.
- Biopsy confirmation is frequently essential for diagnosis and management planning.
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.
