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无功能性肾上腺皮质腺瘤:一例同时合并骨髓脂肪瘤和血管内皮囊肿的罕见病例。

Non-functional adrenocortical adenoma: a unique case of combination with myelolipoma and endothelial cysts.

机构信息

Department of Pathology and Cell Biology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807-8555, Japan.

出版信息

Pathol Res Pract. 2011 Mar 15;207(3):192-6. doi: 10.1016/j.prp.2010.07.008. Epub 2010 Sep 16.

DOI:10.1016/j.prp.2010.07.008
PMID:20846791
Abstract

A case of non-functioning adrenocortical adenoma combined with myelolipoma and endothelial cysts is reported. A 72-year-old Japanese female was noticed to have right renal and left adrenal tumors by an abdominal CT scan. At surgery, the mildly enlarged left adrenal gland contained a well-demarcated tumor. Macroscopically, it was yellowish to dark red or grayish in color, and was characterized by geographic appearance on the cut surface. Histopathological examination revealed a solid proliferation of clear or compact cells and a normal rim of adrenal gland, coexisting with vascular multiple cysts and myelolipomas. The cysts were filled with clotted blood, fibrinous material, or thrombi, and were partially lined with flattened endothelial cells with focal papillary hyperplasia, which were immunohistochemically positive for CD31 and CD34. These cystic walls were often thickened with hyalinized fibrosis and calcification, and were connected to myelolipomatous elements. To our knowledge, this is the first case report of adrenocortical adenoma associated with myelolipoma and endothelial cysts. It is probable that the extensive degeneration in adenoma might induce myelolipomatous metaplasia and cystic vascular formation.

摘要

报告 1 例无功能肾上腺皮质腺瘤合并骨髓脂肪瘤和血管内皮囊肿。1 例 72 岁日本女性因腹部 CT 扫描发现右肾和左肾上腺肿瘤而就诊。手术时,轻度增大的左肾上腺含有界限清楚的肿瘤。大体上,肿瘤呈黄红色至暗红色或灰白色,切面呈地图样外观。组织病理学检查显示,透明细胞或致密细胞的实性增生和正常的肾上腺边缘共存,伴有血管多发囊肿和骨髓脂肪瘤。囊肿内充满凝固的血液、纤维蛋白物质或血栓,部分被扁平的内皮细胞衬里,伴有局灶性乳头状增生,免疫组化 CD31 和 CD34 阳性。这些囊壁常伴有玻璃样纤维化和钙化增厚,并与骨髓脂肪瘤成分相连。据我们所知,这是首例报道的与骨髓脂肪瘤和血管内皮囊肿相关的肾上腺皮质腺瘤。广泛的腺瘤变性可能诱导骨髓脂肪瘤化生和囊状血管形成。

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