Abdominal Extrarenal Involvement in Erdheim-Chester Disease in a Cohort of 304 Patients
Open Access
- 1 December 2022
- journal article
- letter
- Published by American Medical Association (AMA) in JAMA Oncology
- Vol. 8 (12) , 1843-1845
Abstract
Erdheim-Chester disease (ECD) is a multisystem tissue accumulation of foamy histiocytes with constant activation of the mitogen-activated protein kinase pathway leading to organ dysfunction. Specific imaging features, such as perirenal fat stranding, bilateral osteosclerosis of the long bones, parietal aortic-arch sheathing, and sclerosing facial sinuses are well described, though very few descriptions of the involvement of abdominal organs outside the retroperitoneum are reported.1-3 The aim of this study was to estimate the prevalence of nonretroperitoneal abdominal organ involvement in ECD in a large cohort of patients.Keywords
This publication has 6 references indexed in Scilit:
- Multisystemic Imaging Findings in Chinese Patients With Erdheim-Chester DiseaseAmerican Journal of Roentgenology, 2019
- 18F-FDG PET/CT in Erdheim–Chester Disease: Imaging Findings and Potential BRAF Mutation BiomarkerJournal of Nuclear Medicine, 2017
- Mesenteric panniculitis: Still an ambiguous conditionDiagnostic and Interventional Imaging, 2015
- Unusual manifestation of Erdheim-Chester diseaseBMC Gastroenterology, 2011
- Atteinte mésentérique d’une maladie d’Erdheim-Chester : une localisation rareJournal de Radiologie, 2010
- Erdheim-Chester disease with predominant mesenteric localization: Lack of efficacy of interferon alphaJoint Bone Spine, 2009