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异位促肾上腺皮质激素综合征

Ectopic ACTH syndrome.

作者信息

Del Gaudio A

机构信息

Department of Surgery, University of Bologna, Italy.

出版信息

Int Surg. 1988 Jan-Mar;73(1):44-9.

PMID:3360577
Abstract

The recognition of extrapituitary ACTH-secreting tumors is of great importance, not only theoretically but also in practice, in that it permits rational and radical therapy of the relative Cushing's syndrome and avoids more traumatic and risky surgery such as the destruction of the hypophysis and total adrenalectomy. In some cases, however, a systematic search for these tumors, which can be extremely small, is unsuccessful even when the most modern diagnostic tools are employed. Thus, it is still sometimes necessary to resort to total adrenalectomy for provisional control of the Cushing's syndrome, delaying definitive treatment until the tumor makes itself evident. Two clinical cases are described in which, although elements suggesting the possibility of an ectopic neoplasia were present at the time of adrenalectomy, they were not recognized as such, and the tumor responsible for the syndrome was not identified until many years later.

摘要

识别垂体外分泌促肾上腺皮质激素(ACTH)的肿瘤不仅在理论上而且在实践中都非常重要,因为它能对相关的库欣综合征进行合理且彻底的治疗,避免诸如垂体破坏和全肾上腺切除术等更具创伤性和风险的手术。然而,在某些情况下,即便使用了最先进的诊断工具,对这些可能极小的肿瘤进行系统排查仍可能无果。因此,有时仍有必要进行全肾上腺切除术来临时控制库欣综合征,将确定性治疗推迟到肿瘤显现出来。本文描述了两例临床病例,尽管在肾上腺切除术时存在提示异位肿瘤可能性的因素,但当时未被识别,直到多年后才确定导致该综合征的肿瘤。

相似文献

1
Ectopic ACTH syndrome.异位促肾上腺皮质激素综合征
Int Surg. 1988 Jan-Mar;73(1):44-9.
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[Malignant carcinoid tumor of the lungs secreting ectopic ACTH and secondary Cushing's syndrome in a 9-year-old boy].
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[Ectopic ACTH syndrome].[异位促肾上腺皮质激素综合征]
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[Occult ectopic Cushing's syndrome].[隐匿性异位库欣综合征]
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