ISSN: 1899-0967
Polish Journal of Radiology
Established by prof. Zygmunt Grudziński in 1926 Sun
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2018
vol. 83
 
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abstract:
Review paper

Imaging findings in Erdheim-Chester disease: what every radiologist needs to know

Pawan Kumar
,
Anuradha Singh
,
Shivanand Gamanagatti
,
Sanjeev Kumar
,
Sheragaru Hanumanthappa Chandrashekhara

© Pol J Radiol 2018; 83: e54-e62
Online publish date: 2018/02/04
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Erdheim-Chester disease (ECD) is a rare sporadic non-Langerhans cell histiocytic (LCH) proliferative disorder with systemic predilection. It usually affects adults in the 5th-7th decades of life and has non-specific clinical manifestations. Its suspicion is often heralded by the presence of characteristic radiological findings and subsequently confirmed by demonstration of CD68-positive xanthogranulomatous infiltrates on histopathology. Despite being a non-malignant entity, it might be fatal due to organ dysfunction. Imaging plays a key role in the diagnosis, management, and follow-up. Imaging findings are essential to establish the diagnosis, assess actual disease burden, and explore the aetiopathogenesis and therapeutic options to halt disease progression and associated morbidity.
keywords:

diabetes insipidus, neurogenic, Erdheim-Chester disease, non-Langerhans cell histiocytosis




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