Extra-osseous involvement of Langerhans' cell histiocytosis in children

Pediatr Radiol. 2004 Apr;34(4):313-21. doi: 10.1007/s00247-003-1118-z. Epub 2004 Jan 23.


The predominant clinical and radiological features of Langerhans' cell histiocytosis (LCH) in children are due to osseous involvement. Extra-osseous disease is far less common, occurring in association with bone disease or in isolation; nearly all anatomical sites may be affected and in very various combinations. The following article is based on a multicentre review of 31 children with extra-osseous LCH. The objective is to summarise the diverse possibilities of organ involvement. The radiological manifestations using different imaging modalities are rarely pathognomonic on their own. Nevertheless, familiarity with the imaging findings, especially in children with systemic disease, may be essential for early diagnosis.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Age Distribution
  • Central Nervous System Diseases / diagnosis
  • Central Nervous System Diseases / epidemiology*
  • Child
  • Child, Preschool
  • Comorbidity
  • Connective Tissue Diseases / diagnosis
  • Connective Tissue Diseases / epidemiology*
  • Diagnostic Imaging / methods
  • Digestive System Diseases / diagnosis
  • Digestive System Diseases / epidemiology*
  • Female
  • Histiocytosis, Langerhans-Cell / diagnosis*
  • Histiocytosis, Langerhans-Cell / epidemiology*
  • Humans
  • Incidence
  • Infant
  • Male
  • Prognosis
  • Risk Assessment
  • Severity of Illness Index
  • Sex Distribution