Ishida Atsushi, Asakuno Keizoh, Shiramizu Hideki, Yoshimoto Haruko, Nakase Ko, Kato Masataka, Hirayama Masahiro, Sato Hikari, Matsuo Seigo, Miki Nobuhiro, Ono Masami, Yamada Shozo
Department of Neurosurgery, Moriyama Memorial Hospital, Tokyo 134-0088, Japan.
Hypothalamic & Pituitary Center, Moriyama Neurological Center Hospital, Tokyo 134-0081, Japan.
Endocr J. 2021 Oct 28;68(10):1217-1223. doi: 10.1507/endocrj.EJ21-0156. Epub 2021 May 28.
Cushing's disease (CD), which manifests as excess cortisol secretion, is caused by adrenocorticotrophic hormone (ACTH)-secreting pituitary adenomas. Such adenomas are occasionally difficult to identify on magnetic resonance imaging (MRI), and thorough endocrinological examination may be required to detect them. Inferior petrosal sinus (IPS) sampling (IPSS) has been the gold standard test for distinguishing CD from ectopic ACTH syndrome (EAS). However, IPSS is an invasive procedure, and proper catheterization is occasionally challenging due to anatomical variations. Thus, there have been ongoing debates regarding the necessity of this procedure. Here, we present our recent IPSS data derived from the analysis of 65 patients who were referred to us for possible CD between April 2018 and December 2020 after undergoing meticulous endocrinological testing. Even with detailed MRI, no obvious lesions were identified in 19 patients. IPSS performed on these 19 individuals identified an IPS-to-peripheral ACTH gradient in 15 of them. The four patients who lacked this gradient were subjected to a classical algorithm using concurrently measured prolactin levels, the results of which were consistent with their ultimately confirmed diagnoses: two true-positive and two true-negative diagnoses. These findings support the validity of the algorithm and demonstrate that the prolactin-adjusted IPS-to-peripheral ACTH ratio can improve the differentiation between CD and EAS. We had no false-negative results, but three patients were false-positive. Consequently, those three patients in which no apparent tumor was clarified during surgery could not have any endocrinological improvement postoperatively.
库欣病（CD）表现为皮质醇分泌过多，由分泌促肾上腺皮质激素（ACTH）的垂体腺瘤引起。此类腺瘤在磁共振成像（MRI）上有时难以识别，可能需要进行全面的内分泌检查来检测它们。岩下窦（IPS）采样（IPSS）一直是区分CD与异位ACTH综合征（EAS）的金标准检查。然而，IPSS是一种侵入性操作，由于解剖变异，有时正确插管具有挑战性。因此，关于该操作的必要性一直存在争议。在此，我们展示了我们最近的IPSS数据，这些数据来自于对2018年4月至2020年12月期间因可能患有CD而转诊至我们这里的65例患者进行细致内分泌检查后的分析。即使进行了详细的MRI检查，仍有19例患者未发现明显病变。对这19例患者进行的IPSS检查发现其中15例存在IPS与外周ACTH梯度。缺乏该梯度的4例患者采用同时测量催乳素水平的经典算法，其结果与最终确诊结果一致：2例假阳性和2例假阴性诊断。这些发现支持了该算法的有效性，并表明经催乳素校正的IPS与外周ACTH比值可改善CD与EAS之间的鉴别。我们没有假阴性结果，但有3例假阳性。因此，这3例在手术中未明确发现明显肿瘤的患者术后内分泌功能未得到改善。
