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

Frontiers in Oncology

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

[1] Department of Radiology, The Second Affiliated Hospital of Soochow University, Suzhou, China;Department of Radiology, Zhongda Hospital, Medical School of Southeast University, Nanjing, China;

关键词: Erdheim-Chester disease;    non-Langerhans cell histiocytosis;    intestinal obstruction;    bone imaging;    oncology;   

DOI  :  10.3389/fonc.2022.849578

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown