Clinical and Diagnostic Imaging Manifestations of Erdheim-Chester Disease

Authors

Avrey Thau, Wright State UniversityFollow
Roland Gazaille
Alejandro Calvo, Wright State UniversityFollow
Christina E. Borchers, Wright State University - Main CampusFollow
Ronald E. Warwar, Wright State University - Main CampusFollow
William Boyce
Joseph Blake

Document Type

Article

Publication Date

3-23-2021

Abstract

Introduction: Erdheim-Chester disease (ECD) is a rare form of non-Langerhans-cell histiocytosis which typically presents with bilateral masses due to retro-orbital deposition, xanthelasma of the eyelids, cardiopulmonary manifestations, along with sclerosis and cortical thickening of the long bones, particularly in the lower extremities.

Case Report: We present the case of a 58-year-old male who presented to a local emergency department with a one day history of severe dizziness, diaphoresis, dyspnea, and intermittent diplopia. Imaging demonstrated bilateral symmetric retrobulbar masses. Subsequent imaging as well as orbital and bone marrow biopsies lead to the diagnosis of ECD.

Conclusion: Erdheim-Chester disease is a rare form of non-Langerhans-cell histiocytosis. This case demonstrates classic clinical and imaging findings representative of ECD. Diagnostic imaging and pathologic findings play a vital role in the diagnosis and choice of therapy in patients with ECD. Although interferon is currently considered to be first-line therapy, targeted Avrey Thau1, MD, Roland Gazaille2, DO, Alejandro Calvo3, MD, Christina E Borchers4, MS, Ronald Warwar5, MD, William Boyce2, MD, Joseph Blake2, MD Affiliations: 1Department of Internal Medicine, Kettering Health Network, Kettering, Ohio, USA; 2Department of Diagnostic Radiology, Kettering Health Network, Kettering, Ohio, USA; 3Kettering Cancer Center, Division of Medical Oncology and Hematology, Kettering, Ohio, USA; 4Wright State University Boonshoft School of Medicine, Dayton, Ohio, USA; 5Department of Ophthalmology, Kettering Health Network, Kettering, Ohio, USA. Corresponding Author: Roland Gazaille, DO, Department of Diagnostic Radiology, Kettering Health Network, Kettering, Ohio, USA; Email: roland.gazaille@ketteringhealth.org Received: 15 September 2020 Accepted: 05 February 2021 Published: 23 March 2021 PEER REVIEWED | OPEN ACCESS BRAF and MEK inhibitors hold promise for future direction.

Comments

This work is licensed under a Creative Commons Attribution 4.0 International License.

Repository Citation

Thau, A., Gazaille, R., Calvo, A., Borchers, C. E., Warwar, R. E., Boyce, W., & Blake, J. (2021). Clinical and Diagnostic Imaging Manifestations of Erdheim-Chester Disease. Journal of Case Reports and Images in Oncology, 7.
https://corescholar.libraries.wright.edu/dermatology/7

DOI

10.5348/100079Z10AT2021CR