دورية أكاديمية

Erdheim-Chester disease: an unusual aetiology of bilateral lipomatous perinephric masses.

التفاصيل البيبلوغرافية
العنوان: Erdheim-Chester disease: an unusual aetiology of bilateral lipomatous perinephric masses.
المؤلفون: Singh R; Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India., Naranje P; Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India priyanka11sh@gmail.com., Ramateke P; Pathology, All India Institute of Medical Sciences, New Delhi, India., Damle NA; Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.
المصدر: BMJ case reports [BMJ Case Rep] 2021 Apr 07; Vol. 14 (4). Date of Electronic Publication: 2021 Apr 07.
نوع المنشور: Case Reports; Journal Article
اللغة: English
بيانات الدورية: Publisher: BMJ Pub. Group Country of Publication: England NLM ID: 101526291 Publication Model: Electronic Cited Medium: Internet ISSN: 1757-790X (Electronic) Linking ISSN: 1757790X NLM ISO Abbreviation: BMJ Case Rep Subsets: MEDLINE
أسماء مطبوعة: Original Publication: London : BMJ Pub. Group
مواضيع طبية MeSH: Erdheim-Chester Disease*/diagnosis , Erdheim-Chester Disease*/diagnostic imaging, Histiocytes ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Positron-Emission Tomography ; Tomography, X-Ray Computed
مستخلص: A 53-year-old man presented with a history of progressive abdominal distention for 1 year. Physical examination revealed large palpable masses in the bilateral flank regions. Contrast-enhanced CT of the abdomen showed bilateral, symmetrical large perinephric masses with fat attenuating areas, which was further confirmed on MRI. CT of the paranasal sinuses revealed circumscribed extraconal soft tissue mass in the left orbit, causing scalloping and erosion of the left orbital roof. Fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography/CT showed FDG uptake in the bilateral perinephric masses. Based on imaging appearance, a diagnosis of Erdheim-Chester disease (ECD) was suggested. Ultrasound-guided biopsy from perinephric masses revealed a sheet of histiocytes with sprinkled lymphocytes and plasma cells in the background. The histiocytes were immunopositive for CD68, S100 and immunonegative for CD1a, which confirmed the diagnosis of ECD. The patient was started on interferon-α-2a and showed symptomatic improvement.
Competing Interests: Competing interests: None declared.
(© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)
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فهرسة مساهمة: Keywords: haematology (incl blood transfusion); pathology; radiology
تواريخ الأحداث: Date Created: 20210408 Date Completed: 20210514 Latest Revision: 20230408
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC8030666
DOI: 10.1136/bcr-2020-239137
PMID: 33827868
قاعدة البيانات: MEDLINE
الوصف
تدمد:1757-790X
DOI:10.1136/bcr-2020-239137