AUTHOR=Zhou Xiuzhi , Zhai Duchang , Yang Junlin , Shi Dai , Lu Kuan , Cai Wu , Fan Guohua , Ju Shenghong 

TITLE=A Patient With Erdheim-Chester Disease Presenting With Intestinal Obstruction as the Initial Symptom: A Case Report

JOURNAL=Frontiers in Oncology

VOLUME=Volume 12 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2022.849578

DOI=10.3389/fonc.2022.849578

ISSN=2234-943X

ABSTRACT=Erdheim-Chester disease (ECD) is a rare and systemic non-Langerhans cell histiocytosis. Recently ECD was classified as an inflammatory medullary tumor that affects a diverse group of organ systems. The purpose of this report is to present the radiological features of this disease in a 51-year-old man with intestinal obstruction as the initial presentation. In this case, CT and ECT clearly showed the lesions of various systems, especially the skeletal images. The survival benefit of treatment with interferon -α (IFN-α) and BRAF inhibitors is well established, while the rest of the treatment focuses on symptom relief.