. 2019; 28(1): 0-0

A diagnostic challenge: Erdheim Chester disorder

Mairah Razi1, Maria Qubtia2, Aamna Hassan1, Mudassar Hussain3, Abdul Hameed2
1Department Of Nuclear Medicine, Shaukat Khanum Memorial Cancer Hospital And Research Centre, Lahore, Pakistan
2Department Of Medical Oncology, Shaukat Khanum Memorial Cancer Hospital And Research Centre, Lahore, Pakistan
3Department Of Pathology, Shaukat Khanum Memorial Cancer Hospital And Research Centre, Lahore, Pakistan

Erdheim Chester Disease is a rare, multisystem, idiopathic disease often associated with BRAF V600E. Diagnosis is typically delayed and challenging because of variable manifestations. Although it has an indolent course; advanced stages can show fulminant behavior due to multiple vital organ involvement. It is a class IIa, non-Langerhans cells histiocytosis with characteristic radiological appearance. Whole body imaging will be more helpful, particularly, to assess skeletal lesions. Widespread disease with typical skeletal involvement on imaging can prompt this diagnosis. However, histopathology with immunohistochemistry is needed to confirm the diagnosis. Disease can also manifest itself with a large variety of CNS related or orbital symptoms. Cardiac involvement is quite common.
We present an interesting image of a patient with Erdheim Chester disease who underwent positron emission tomography – computed tomography (PET-CT).
Informed consent of subject described in this image is waived by the institutional review board.

Keywords: Erdheim Chester Disease, Non Langerhans cell histiocytosis, Positron emission tomography


Mairah Razi, Maria Qubtia, Aamna Hassan, Mudassar Hussain, Abdul Hameed. A diagnostic challenge: Erdheim Chester disorder. . 2019; 28(1): 0-0

Corresponding Author: Mairah Razi, Pakistan


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