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Erdheim-Chester disease: a rare non-Langerhans histiocytosis
  1. Sofia Costa1,
  2. Maria José Julião2,
  3. Sónia Silva1 and
  4. Manuel João Brito1
  1. 1Pediatric Oncology, Centro Hospitalar e Universitario de Coimbra EPE, Hospital Pediátrico, Coimbra, Portugal
  2. 2Department of Pathology, Coimbra University Hospital, Coimbra, Portugal
  1. Correspondence to Dr Sofia Costa; sofiacosta89{at}


A 3-year-old girl was admitted to our hospital with diabetes insipidus and a left eye proptosis. During investigation of diabetes insipidus, an extensive osteolytic mass, involving skull base and maxillo-facial bones, was revealed. Biopsy exhibited dense infiltrate of foamy histiocytes, which were positive for CD68 and CD163 and negative for CD1a and S100 confirming histopathological diagnosis of Erdheim-Chester disease. Treatment with dabrafenib was initiated, with good response and no side effects.

  • paediatric oncology
  • head and neck cancer

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  • Contributors SC wrote the manuscript. SS and MJB reviewed the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.