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Updated: Jun 15, 2025

Imaging CD4 T Cell Interstitial Migration in the Inflamed Dermis

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Published on: March 25, 2016

Imaging in Erdheim-Chester Disease.

Yashant Aswani¹, Aditi Patel¹, Xin Zhan¹

¹From the Department of Radiology, University of Iowa Hospitals & Clinics, 200 Hawkins Dr, 3882 John Pappajohn Pavilion, Iowa City, IA 52242 (Y.A., A.P., X.Z., S.K., S. Averill); Department of Radiology, Rush University Medical Center, Chicago, Ill (S. Ansari); Department of Radiology, Radiology Institute of University of São Paulo, São Paulo, Brazil (L.G.M.); Department of Neurology, Geetanjali Medical College and Hospital, Udaipur, India (N.A.); Department of Radiology, Seth GS Medical College and KEM Hospital, Mumbai, India (D.D.P.); and Department of Radiology, Mayo Clinic, Jacksonville, Fla (S.B.).

Radiographics : a Review Publication of the Radiological Society of North America, Inc

|August 22, 2024

Summary

This summary is machine-generated.

Erdheim-Chester disease (ECD) is a rare neoplastic process now understood to involve the MAPK pathway. Radiology plays a crucial role in diagnosing ECD, assessing its extent, and guiding management strategies.

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Area of Science:

Oncology
Histiocytic Neoplasms
Radiology

Background:

Erdheim-Chester disease (ECD) is a rare, multisystemic, non-Langerhans cell histiocytic neoplasm.
Recurrent mutations in the MAPK pathway, particularly BRAF, have reclassified ECD as a neoplastic process.
ECD presents with diverse clinical symptoms including bone pain, fatigue, and neurological manifestations.

Purpose of the Study:

To review the evolving role of radiology in the diagnosis and management of Erdheim-Chester disease.
To discuss radiologic findings and differential diagnoses of ECD using an organ system-based approach.
To outline revised consensus recommendations for ECD evaluation, diagnosis, and treatment.

Main Methods:

Comprehensive review of radiologic findings associated with Erdheim-Chester disease.
Analysis of differential diagnoses based on imaging characteristics across various organ systems.
Integration of molecular pathogenesis insights and updated management guidelines.

Main Results:

Radiologic findings are critical for early detection due to variable clinical and histologic presentations of ECD.
Imaging aids in determining the extent of disease and can inform prognosis.
Targeted therapies like vemurafenib and cobimetinib are emerging, necessitating revised management guidelines.

Conclusions:

Radiology is indispensable for diagnosing Erdheim-Chester disease, assessing its extent, and guiding prognosis.
An organ system-based approach aids in interpreting diverse radiologic findings and differential diagnoses.
Revised consensus recommendations emphasize a multidisciplinary approach to ECD management, incorporating molecular insights and targeted therapies.