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A diagnostic challenge: Erdheim Chester disorderMairah Razi1, Maria Qubtia2, Aamna Hassan1, Mudassar Hussain3, Abdul Hameed21Department Of Nuclear Medicine, Shaukat Khanum Memorial Cancer Hospital And Research Centre, Lahore, Pakistan2Department 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. 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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