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

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

doi:10.3389/fonc.2022.849578

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

Front Oncol. 2022 Mar 16:12:849578. doi: 10.3389/fonc.2022.849578. eCollection 2022.

Authors

Xiuzhi Zhou¹, Duchang Zhai¹, Junlin Yang¹, Dai Shi¹, Kuan Lu¹, Wu Cai¹, Guohua Fan¹, Shenghong Ju²

Affiliations

¹ Department of Radiology, The Second Affiliated Hospital of Soochow University, Suzhou, China.
² Department of Radiology, Zhongda Hospital, Medical School of Southeast University, Nanjing, China.

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, X-ray computed tomography (CT) and emission computed tomography (ECT) clearly showed lesions in various systems, especially in the skeletal images. The survival benefit of treatment with interferon α (IFN-α) and BRAF inhibitors is well established, while other treatments focus on symptom relief.

Keywords: Erdheim-Chester disease; bone imaging; intestinal obstruction; non-Langerhans cell histiocytosis; oncology.

Publication types

Case Reports