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双侧肾上腺髓质脂肪瘤伴库欣综合征

Bilateral adrenal myelolipomas with Cushing's syndrome.

作者信息

Kanj H A, Noronha J, D'Aguillo A F, Amorosa J K, Amorosa L F

机构信息

Department of Medicine, UMDNJ-Robert Wood Johnson Medical School, New Brunswick 08903-0019.

出版信息

JAMA. 1988 May 27;259(20):3034-6.

PMID:2835522
Abstract

A 24-year-old woman with non-pituitary dependent Cushing's syndrome was found to have bilateral adrenal myelolipomas at surgery. These benign tumors consist of bone marrow and fat and are uncommon incidental findings that are discovered by computed tomography. Myelolipomas have rarely been associated with Cushing's syndrome, but the cause of the syndrome has not always been defined, because the reports preceded modern diagnostic methods. Careful examination of the adrenal glands from our patient showed a fusion of myelolipoma elements and adrenal cells without distinct adenomas or the typical nodular pattern of adrenal hyperplasia. This report suggests that adrenal myelolipomas and atypical hyperplasia of the zona fasciculata may be anatomically and functionally related.

摘要

一名患有非垂体依赖性库欣综合征的24岁女性在手术中被发现双侧肾上腺髓质脂肪瘤。这些良性肿瘤由骨髓和脂肪组成,是通过计算机断层扫描偶然发现的罕见情况。髓质脂肪瘤很少与库欣综合征相关,但由于相关报告早于现代诊断方法,该综合征的病因并不总是明确的。对我们患者的肾上腺进行仔细检查发现,髓质脂肪瘤成分与肾上腺细胞融合,没有明显的腺瘤或肾上腺增生的典型结节模式。本报告表明,肾上腺髓质脂肪瘤与束状带非典型增生可能在解剖学和功能上相关。

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