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分泌醛固酮的肾上腺皮质癌。病例报告及文献综述。

Aldosterone-secreting adrenal cortical carcinoma. A case report and review of the literature.

作者信息

Griffin Adrienne Carruth, Kelz Rachel, LiVolsi Virginia A

机构信息

Department of Pathology, Passavant Hospital, University of Pittsburgh Medical Center, 9100 Babcock Blvd, Pittsburgh, PA, 15237, USA,

出版信息

Endocr Pathol. 2014 Sep;25(3):344-9. doi: 10.1007/s12022-014-9307-x.

Abstract

Adrenal cortical carcinomas (ACC) are rare, typically aggressive malignant neoplasms with a reported incidence of 1-2 cases per 1 million population and account for 0.05-0.2 % of all malignancies. The majority of these tumors are functional with approximately 60 % of patients experiencing endocrine symptomatology typically characterized by Cushing's syndrome (40 %) or a mixed hormonal picture of Cushing syndrome seen in association with virilization. Rarely, patients present with a pure hormonal syndrome of feminization or hyperaldosteronism, 6 and 2.5 %, respectively. We report a case of a 76-year-old woman presenting with recently diagnosed hypertension secondary to primary hyperaldosteronism. The patient underwent laparoscopic converted to an open adrenalectomy and a diagnosis of adrenocortical carcinoma (aldosteronoma clinical) was rendered. This case and review of the literature highlight that while rare, aldosterone-secreting adrenal cortical carcinomas may occur. In this case report, we discuss the clinical presentation, pathologic findings, and review the literature for adrenal cortical carcinomas and aldosterone-secreting adrenal cortical carcinomas.

摘要

肾上腺皮质癌(ACC)较为罕见,通常是侵袭性恶性肿瘤,报告发病率为每100万人口中有1 - 2例,占所有恶性肿瘤的0.05 - 0.2%。这些肿瘤大多具有功能性,约60%的患者有内分泌症状,典型表现为库欣综合征(40%)或与男性化相关的库欣综合征混合激素表现。很少有患者分别表现为单纯的女性化或醛固酮增多症激素综合征,比例分别为6%和2.5%。我们报告一例76岁女性患者,因原发性醛固酮增多症继发近期诊断的高血压。该患者接受了腹腔镜转为开放肾上腺切除术,诊断为肾上腺皮质癌(临床醛固酮瘤)。该病例及文献回顾强调,虽然分泌醛固酮的肾上腺皮质癌罕见,但仍可能发生。在本病例报告中,我们讨论了临床表现、病理结果,并回顾了肾上腺皮质癌和分泌醛固酮的肾上腺皮质癌的文献。

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