背景:库欣病(CD)是皮质醇增多症最常见的病因之一。磁共振成像(MRI)通常用于CD的诊断。然而,高达64%的促肾上腺皮质激素(ACTH)产生的垂体微腺瘤在MRI上检测不到.我们报告了15例MRI阴性CD,他们采用单纯的内镜经鼻入路进行了手术切除。
方法:由一名外科医生对134例CD患者进行了鼻内镜经蝶入路手术(EETS)。15例符合纳入标准:没有结论性的MRI研究,也没有以前的手术治疗。收集的数据包括体征/症状,术前和术后激素水平,以及手术或医疗管理引起的并发症。有关肿瘤直径的数据,location,和肿瘤残留/复发从术前和术后MRI获得。进行免疫组织化学以评估肿瘤激素分泌。
结果:除了MRI阴性和阳性患者的组织病理学结果有统计学上的显着差异(P=0.001),在其他人口统计学或临床数据点,两组间无统计学显著差异.对15例MRI不确定的患者进行了去氨加压素(DDAVP®)给药的岩下窦采样(IPSS),以通过中枢/外周(C/P)比率确定ACTH分泌过多的起源。IPSS在七个,五名和三名患者显示正确,左,和中央侧偏侧化,分别。平均随访5.5年,在MRI阴性患者中,14例(93%)和12例(80%)患者实现了早期和长期缓解,分别。在MRI阳性队列中,在平均4.8年的随访中,113名患者(94.9%)和102名患者(85.7%)实现了初始和长期缓解,分别。
结论:MRI阴性/不确定的库欣病的外科治疗是具有挑战性的方案,需要多学科方法。一位经验丰富的神经外科医生,与专门的内分泌学家合作,应利用IPSS发现确定腺瘤的最可能位置,随后对垂体进行仔细的手术探查以确定腺瘤。
BACKGROUND: Cushing\'s disease (CD) is among the most common etiologies of hypercortisolism. Magnetic resonance imaging (MRI) is often utilized in the diagnosis of CD, however, up to 64% of adrenocorticotropic hormone (ACTH)-producing pituitary microadenomas are undetectable on MRI. We report 15 cases of MRI negative CD who underwent surgical resection utilizing a purely endoscopic endonasal approach.
METHODS: Endoscopic endonasal transsphenoidal surgery (EETS) was performed on 134 CD cases by a single surgeon. Fifteen cases met inclusion criteria: no conclusive MRI studies and no previous surgical treatment. Data collected included signs/symptoms, pre- and post-operative hormone levels, and complications resulting from surgical or medical management. Data regarding tumor diameter, location, and tumor residue/recurrence was obtained from both pre- and post-operative MRI. Immunohistochemistry was performed to assess for tumor hormone secretion.
RESULTS: Aside from a statistically significant difference (P = 0.001) in histopathological results between patients with negative and positive MRI, there were no statistically significant difference between these two groups in any other demographic or clinical data point. Inferior petrosal sinus sampling (IPSS) with desmopressin (DDAVP®) administration was performed on the 15 patients with inconclusive MRIs to identify the origin of ACTH hypersecretion via a central/peripheral (C/P) ratio. IPSS in seven, five and three patients showed right, left, and central side lateralization, respectively. With a mean follow-up of 5.5 years, among MRI-negative patients, 14 (93%) and 12 patients (80%) achieved early and long-term remission, respectively. In the MRI-positive cohort, over a mean follow-up of 4.8 years, 113 patients (94.9%) and 102 patients (85.7%) achieved initial and long-term remission, respectively.
CONCLUSIONS: Surgical management of MRI-negative/inconclusive Cushing\'s disease is challenging scenario requiring a multidisciplinary approach. An experienced neurosurgeon, in collaboration with a dedicated endocrinologist, should identify the most likely location of the adenoma utilizing IPSS findings, followed by careful surgical exploration of the pituitary to identify the adenoma.