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肾上腺巨大囊性嗜铬细胞瘤。

A giant cystic pheochromocytoma of the adrenal gland.

作者信息

Pan Zenggang, Repertinger Susan, Deng Caishu, Sharma Poonam

机构信息

Department of Pathology, Creighton University Medical Center, 601 N. 30th Street, Omaha, NE 68131, USA.

出版信息

Endocr Pathol. 2008 Summer;19(2):133-8. doi: 10.1007/s12022-008-9016-4.

Abstract

Adrenal pheochromocytomas are rare catecholamine-secreting tumors that originate from chromaffin cells in the adrenal medulla, and giant pheochromocytomas with cystic changes are particularly rare. We report a case of a 46-year-old man who presented with episodic hypertension and headache. Radiographic studies showed an 18-cm cystic mass in the left upper quadrant of the abdomen; further workups, which included light microscopy, immunohistochemical, and electron microscopic analysis, revealed a pheochromocytoma of the left adrenal gland. Cytogenetic analysis and genetic mutation analyses for von-Hippel-Lindau (VHL), rearranged during transfection (RET), and succinate dehydrogenase complex subunit B (SDHB) genes were also performed but failed to reveal any abnormalities within the tumor cells.

摘要

肾上腺嗜铬细胞瘤是一种罕见的分泌儿茶酚胺的肿瘤,起源于肾上腺髓质的嗜铬细胞,而伴有囊性变的巨大嗜铬细胞瘤则尤为罕见。我们报告一例46岁男性患者,其表现为发作性高血压和头痛。影像学检查显示腹部左上象限有一个18厘米的囊性肿块;进一步检查,包括光学显微镜检查、免疫组织化学和电子显微镜分析,发现是左肾上腺嗜铬细胞瘤。还进行了针对冯-希佩尔-林道(VHL)、转染期间重排(RET)和琥珀酸脱氢酶复合物亚基B(SDHB)基因的细胞遗传学分析和基因突变分析,但未发现肿瘤细胞内有任何异常。

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