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阴道小细胞癌通过异位产生和分泌促肾上腺皮质激素导致库欣综合征:一例报告

Small cell carcinoma of the vagina causing Cushing's syndrome by ectopic production and secretion of ACTH: a case report.

作者信息

Colleran K M, Burge M R, Crooks L A, Dorin R I

机构信息

Department of Medicine, Albuquerque VA Medical Center and University of New Mexico School of Medicine, 87108, USA.

出版信息

Gynecol Oncol. 1997 Jun;65(3):526-9. doi: 10.1006/gyno.1997.4701.

Abstract

BACKGROUND

Small cell carcinomas of pulmonary or extrapulmonary origin are neuroendocrine tumors classically associated with ectopic hormone production, particularly ACTH secretion resulting in Cushing's syndrome. However, ectopic Cushing's syndrome has not previously been reported in the setting of small cell carcinoma of the vagina.

METHODS

A primary vaginal tumor with hepatic metastases was evaluated with light microscopy. Serum cortisol and plasma ACTH levels were evaluated by radioimmunoassay and immunoradiometric assay, respectively, during a standard high-dose (8 mg) overnight dexamethasone suppression test.

RESULTS

Vaginal small cell carcinoma with hepatic metastases was demonstrated. Electrolyte abnormalities, elevated cortisol and ACTH levels, and failure to suppress ACTH secretion during high-dose dexamethasone administration confirmed the diagnosis of ectopic ACTH syndrome.

CONCLUSIONS

This case report establishes a clinical association between vaginal small cell carcinoma and ectopic Cushing's syndrome, confirming the neuroendocrine potential of this malignancy and features common to small cell neoplasms originating in other sites.

摘要

背景

肺或肺外起源的小细胞癌是神经内分泌肿瘤,经典地与异位激素产生相关,特别是促肾上腺皮质激素(ACTH)分泌导致库欣综合征。然而,以前尚未报道过阴道小细胞癌患者出现异位库欣综合征。

方法

对一例伴有肝转移的原发性阴道肿瘤进行光学显微镜评估。在标准高剂量(8毫克)过夜地塞米松抑制试验期间,分别通过放射免疫分析和免疫放射分析评估血清皮质醇和血浆ACTH水平。

结果

证实为伴有肝转移的阴道小细胞癌。电解质异常、皮质醇和ACTH水平升高,以及高剂量地塞米松给药期间ACTH分泌未被抑制,证实了异位ACTH综合征的诊断。

结论

本病例报告确立了阴道小细胞癌与异位库欣综合征之间的临床关联,证实了这种恶性肿瘤的神经内分泌潜能以及起源于其他部位的小细胞瘤的共同特征。

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