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胰腺受累于厄尔德海姆-切斯特病:一种罕见疾病的罕见表现。

Pancreatic involvement in Erdheim-Chester disease: Rare presentation of a rare disease.

作者信息

Rafiee Moezedin Javad, Taylor Jana, Hickeson Marc, Friedrich Matthias G, Chetrit Michael

机构信息

Department of Medicine and Diagnostic Radiology, McGill University Health Centre, 1001 Blvd, Decarie, Montreal, Québec, H4A3J1 Canada.

Department of Diagnostic Radiology, McGill University Health Centre, 1001 Blvd, Decarie, Montreal, Québec, H4A3J1, Canada.

出版信息

Radiol Case Rep. 2023 Mar 4;18(5):1809-1820. doi: 10.1016/j.radcr.2023.02.012. eCollection 2023 May.

Abstract

Erdheim-Chester disease (ECD) as a rare non-Langerhans histiocytosis has various clinical manifestations. It is characterized histologically by infiltration of every organ, more commonly bone, retroperitoneum, cardiovascular and CNS systems with foamy, lipid -laden macrophage. Pancreatic involvement as a manifestation of this uncommon disease has very rarely been reported. Here we report a 73-year-old woman with ECD and pancreas involvement in CT, MRI and PET scans. We also aim to increase radiologist knowledge about considering ECD as a differential diagnosis for pancreas mass in the appropriate clinical situation.

摘要

Erdheim-Chester病(ECD)作为一种罕见的非朗格汉斯组织细胞增多症,有多种临床表现。其组织学特征为每个器官均有浸润,更常见于骨骼、腹膜后、心血管和中枢神经系统,伴有泡沫状、富含脂质的巨噬细胞。作为这种罕见疾病的一种表现,胰腺受累的报道极为罕见。在此,我们报告一名73岁女性,其在CT、MRI和PET扫描中显示患有ECD且胰腺受累。我们还旨在提高放射科医生在适当临床情况下将ECD视为胰腺肿块鉴别诊断的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2aee/10006724/109df00217e3/gr1.jpg

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