Lad Shivanand P, Patil Chirag G, Laws Edward R, Katznelson Laurence
Department of Neurosurgery, Stanford University School of Medicine, Stanford, California, 94305, USA.
Neurosurg Focus. 2007;23(3):E2. doi: 10.3171/foc.2007.23.3.3.
Cushing's syndrome can present a complex problem of differential diagnosis. Of cases in which hypercortisolemia results from an adrenocorticotropic hormone (ACTH)-dependent process, approximately 80% are due to a pituitary adenoma (Cushing's disease [CD]), 10% are due to adrenal lesions, and the remaining 10% are secondary to ectopic ACTH secretion. For patients with CD, surgical removal of the pituitary adenoma is the treatment of choice. Thus, localization of the source of ACTH secretion is critical in guiding timely treatment decisions. Inferior petrosal sinus sampling (IPSS) is considered to be the gold standard for confirming the origin of ACTH secretion in patients with Cushing's syndrome. The authors present an overview of IPSS--both the technique and its interpretation--as well as a summary of recent studies. A number of other techniques are discussed including sampling from the cavernous sinus, the jugular vein, and multiple sites to aid the diagnosis and lateralization of ACTH-producing pituitary adenomas. Management is best undertaken by a comprehensive multidisciplinary team taking into account the results of all the biochemical and imaging studies available, to provide the best advice in patient treatment decisions.
库欣综合征可能会带来复杂的鉴别诊断问题。在高皮质醇血症由促肾上腺皮质激素(ACTH)依赖性过程引起的病例中,约80%是由垂体腺瘤(库欣病[CD])所致,10%是由肾上腺病变引起,其余10%继发于异位ACTH分泌。对于CD患者,手术切除垂体腺瘤是首选治疗方法。因此,ACTH分泌源的定位对于指导及时的治疗决策至关重要。岩下窦采样(IPSS)被认为是确诊库欣综合征患者ACTH分泌来源的金标准。作者对IPSS进行了概述,包括该技术及其解读,以及近期研究的总结。还讨论了许多其他技术,包括从海绵窦、颈静脉和多个部位采样,以辅助诊断和定位产生ACTH的垂体腺瘤。最好由一个综合的多学科团队进行管理,同时考虑所有可用的生化和影像学研究结果,以便在患者治疗决策中提供最佳建议。