Langerhans cell histiocytosis: differences and similarities in long-term outcome of paediatric and adult patients at a single institutional centre

Abstract

Purpose: This study determined the frequency of clinical features, reactivations, sequelae, mortality, and overall survival (OS) and compared paediatric with adult Langerhans cell histiocytosis (LCH) patients.

Materials and methods: Ninety patients (60 paediatric and 30 adults) with LCH treated during 28 years were analysed retrospectively.

Results: Craniofacial lesion was the most frequent lesion at LCH presentation in children and adults. However, some differences were found. Orbital lesions were more frequent in paediatric than adult patients (P = 0.001). There was a tendency for mandible lesions to be more common in adults than the paediatric group (P = 0.0710). Mucocutaneous lesions were observed in a higher proportion in adults compared to paediatric patients (P = 0.0395). Reactivation episodes (36.8 versus 62.5%) and deaths (10.7 versus 24.0%) occurred in lower proportions in paediatric than adult patients, respectively. The probability of OS in 10 years for both groups was similar (P = 0.137).

Conclusion: The OS was similar in both groups despite clinical differences between paediatric and adult patients, and higher reactivation and death rates in adults.

Keywords: Adults; Children; Diabetes insipidus; Langerhans cells histiocytosis; Lytic bone lesions.