Fomekong Edward, Maiter Dominique, Grandin Cécile, Raftopoulos Christian
Department of Neurosurgery, Cliniques, Cliniques Universitaires Saint Luc Brussels, Université Catholique de Louvain, Belgium.
Clin Neurol Neurosurg. 2009 Jun;111(5):442-9. doi: 10.1016/j.clineuro.2008.12.011. Epub 2009 Feb 5.
Although numerous studies have shown that transsphenoidal surgery is the best initial treatment for Cushing disease offering 59-95% of success, fewer information is available on the long-term outcome in the subgroup of patients harboring ACTH-secreting macroadenomas. The aims of this study were to analyze our 10-year experience with transsphenoidal surgery in Cushing's disease and to examine whether remission rates were different between micro- and macroadenomas.
Forty consecutive patients with proven Cushing's disease (28 microadenomas, 12 macroadenomas [diameter: 10-25 mm], 3 patients with no visible adenoma at MRI) underwent transsphenoidal surgery (TSS) assisted by neuronavigation in our center between 1996 and 2007. The diagnosis was made using standard endocrinological criteria including bilateral inferior petrosal sinus sampling (BIPSS) with CRH stimulation in all patients with discordant or equivocal biochemical and radiological testing. Morning serum cortisol was measured during the first week postoperatively, and a complete endocrine evaluation was made in all patients at 6-8 weeks. Remission at follow-up was defined as a normal postoperative 24-h urinary free cortisol (UFC) or continued need for glucocorticoid hormone replacement after TSS.
Overall, 32/40 patients (80%) were in remission after one or more TSS. Interestingly, a very good remission rate (92%) was observed in the subset of macroadenomas, similar to that found in the group of microadenomas (84%, NS), while no post-surgical remission was observed in the 3 patients with no visible adenoma at MRI (p<0.01). Of the 8 patients not in remission after repeated TSS surgery, 3 underwent radiation therapy and three had bilateral adrenalectomy, allowing remission of their hypercortisolism. There was minor morbidity and no death.
While our overall results are in accordance with other published series, we show here that ACTH-secreting pituitary macroadenomas are usually not associated with a bad outcome, in contrast with patients with no visible adenoma at preoperative MRI.
尽管众多研究表明经蝶窦手术是库欣病的最佳初始治疗方法，成功率为59% - 95%，但关于分泌促肾上腺皮质激素（ACTH）的大腺瘤亚组患者的长期预后的信息较少。本研究的目的是分析我们在库欣病经蝶窦手术方面的10年经验，并探讨微腺瘤和大腺瘤的缓解率是否存在差异。
1996年至2007年期间，40例经证实的库欣病患者（28例微腺瘤，12例大腺瘤[直径：10 - 25 mm]，3例在磁共振成像（MRI）上未发现可见腺瘤）在我们中心接受了神经导航辅助下的经蝶窦手术（TSS）。所有生化和影像学检查结果不一致或不明确的患者均采用标准内分泌学标准进行诊断，包括双侧岩下窦采血（BIPSS）并给予促肾上腺皮质激素释放激素（CRH）刺激。术后第一周测量早晨血清皮质醇，并在6 - 8周时对所有患者进行全面的内分泌评估。随访时的缓解定义为术后24小时尿游离皮质醇（UFC）正常或经蝶窦手术后仍需要糖皮质激素替代治疗。
总体而言，32/40例患者（80%）在接受一次或多次经蝶窦手术后缓解。有趣的是，在大腺瘤亚组中观察到非常高的缓解率（92%），与微腺瘤组（84%，无显著性差异）相似，而3例MRI上未发现可见腺瘤的患者术后未出现缓解（p<0.01）。在重复经蝶窦手术后未缓解的8例患者中，3例接受了放射治疗，3例进行了双侧肾上腺切除术，从而缓解了皮质醇增多症。手术并发症轻微，无死亡病例。
虽然我们的总体结果与其他已发表的系列研究一致，但我们在此表明，与术前MRI未发现可见腺瘤的患者相比，分泌ACTH的垂体大腺瘤通常不会导致不良预后。
