Interesting Image

A Diagnostic Challenge: Erdheim Chester Disorder


  • Mairah Razi
  • Maria Qubtia
  • Aamna Hassan
  • Mudassar Hussain
  • Abdul Hameed

Received Date: 01.03.2018 Accepted Date: 25.09.2018 Mol Imaging Radionucl Ther 2019;28(1):30-33 PMID: 30942060

Erdheim-Chester disease (ECD) is a rare, multisystemic, idiopathic disease often associated with BRAF V600E mutation. Its diagnosis is typically delayed and challenging due to its variable manifestations. Although it has an indolent course, advanced stages can manifest fulminant behavior with multiple vital organ involvement. It is a class 2a, non-Langerhans cell histiocytosis with characteristic radiological appearance. Whole body imaging might be helpful, particularly, to assess skeletal lesions. Although widespread disease with typical skeletal involvement on imaging can prompt diagnosis, histopathology with immunohistochemistry is required for confirmation. The disease can also manifest itself with a large variety of central nervous system related or orbital symptoms. Cardiac involvement is quite common. We present an interesting image of a patient with ECD who underwent PET/CT. Informed consent of the subject described in this image is waived by the Institutional Review Board.

Keywords: Erdheim-Chester disease, non-Langerhans cell histiocytosis, positron emission tomography


Informed Consent: Consent form was filled out by all participants.

Peer-review: Externally and internally peer-reviewed.

Authorship Contributions

Surgical and Medical Practices: M.R., M.Q., A.Has., M.H., A.H., Concept: M.R., M.Q., A.Has., M.H., A.H., Design: M.R., M.Q., A.H., M.H., A.H., Data Collection or Processing: M.R., M.Q., A.Has., M.H., A.H., Analysis or Interpretation: M.R., M.Q., A.Has., M.H., A.H., Literature Search: M.R., M.Q., A.Has., M.H., A.H., Writing: M.R., M.Q., A.Has., M.H., A.H.

Conflict of Interest: No conflict of interest was declared by the authors.

Financial Disclosure: The authors declared that this study received no financial support.


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