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[一例库欣病合并无功能肾上腺肿瘤]

[A case of Cushing's disease associated with a non-functioning adrenal tumor].

作者信息

Ohguni S, Takagi C, Masaki Y, Notsu K, Hori T, Kato Y

机构信息

Department of Medicine, Shimane Medical University, Izumo, Japan.

出版信息

Nihon Naibunpi Gakkai Zasshi. 1992 Dec 20;68(12):1249-56. doi: 10.1507/endocrine1927.68.12_1249.

Abstract

A 52-year-old woman was admitted to our hospital for further examination of central obesity, hypertension and hirsutism suggesting Cushing's syndrome. Hirsutism had been remarkable for two years, and muscle weakness of the lower extremities gradually developed during the past year. CT scan revealed a tumor in the left adrenal gland which was 1 cm in diameter, round, well-circumscribed, homogeneous and not enhanced. Endocrine data disclosed increased urinary 17-OHCS (11.5-16.4 mg/day) and elevated plasma ACTH (125 pg/ml) and cortisol (19 micrograms/dl) with a lack of diurnal rhythm. Administration of the single-dose dexamethasone (1mg) did not suppress plasma cortisol. However, consecutive administration of either 2mg or 8mg of dexamethasone for 2 days suppressed both plasma cortisol and urinary 17-OHCS. Administration of metyrapone raised both urinary 17-OHCS and plasma ACTH levels. Rapid ACTH test resulted in a hyperresponse of plasma cortisol. CRF injection raised plasma ACTH and cortisol. Bilateral adrenal glands were well demonstrated by 19-iodocholesterol (I-131) scintigraphy during the administration of dexamethasone. MRI with Gd-contrast revealed a microadenoma in the sella turcica. With the diagnosis of Cushing's disease, the microadenoma was removed by the transsphenoidal approach and adrenal function was normalized. However, the left adrenal tumor remained on CT scan but was not demonstrated by scintigraphy. These findings indicate that this is a very rare case of Cushing's disease which was associated with an unilateral non-functioning adrenal tumor.

摘要

一名52岁女性因出现提示库欣综合征的向心性肥胖、高血压和多毛症而入院接受进一步检查。多毛症已持续两年,且在过去一年中逐渐出现下肢肌肉无力。CT扫描显示左肾上腺有一个直径1厘米的肿瘤,呈圆形,边界清晰,密度均匀,无强化。内分泌检查数据显示尿17-羟皮质类固醇增加(11.5 - 16.4毫克/天),血浆促肾上腺皮质激素(ACTH)升高(125皮克/毫升),皮质醇升高(19微克/分升),且昼夜节律消失。单剂量地塞米松(1毫克)给药未能抑制血浆皮质醇。然而,连续2天给予2毫克或8毫克地塞米松可抑制血浆皮质醇和尿17-羟皮质类固醇。甲吡酮给药使尿17-羟皮质类固醇和血浆ACTH水平均升高。快速ACTH试验导致血浆皮质醇反应亢进。促肾上腺皮质激素释放因子(CRF)注射使血浆ACTH和皮质醇升高。在给予地塞米松期间,19-碘胆固醇(I-131)闪烁扫描清晰显示双侧肾上腺。钆增强MRI显示蝶鞍内有微腺瘤。诊断为库欣病后,经蝶窦入路切除微腺瘤,肾上腺功能恢复正常。然而,CT扫描显示左肾上腺肿瘤仍然存在,但闪烁扫描未显示。这些发现表明这是一例非常罕见的库欣病病例,伴有单侧无功能肾上腺肿瘤。

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