Hofmann Bernd M, Fahlbusch Rudolf
Department of Neurosurgery, University of Erlangen-Nuremberg, Erlangen, Germany.
Front Horm Res. 2006;34:158-184. doi: 10.1159/000091580.
We evaluate the current role of microsurgery for Cushing's disease (CD) and the efficacy of adjuvant treatment modalities. The standard treatment for primary CD remains transsphenoidal surgery followed by adjuvant therapy in cases with persisting hypercortisolism. Moderately severe cases are treated with radiotherapy, while in the very severe adrenalectomy is performed. In our series of primary CD (March 1997 to September 2004, mean observation period 18.8 months) adenomas were confirmed intraoperatively in 84.0% of the cases. Remission was achieved in 75.0% and recurrence was observed in 4.8% of the patients. Complications occurred in 2.0% of the cases and all resolved without resulting in permanent morbidity. In the literature, the rates of intraoperative confirmation of an adenoma vary between 59.1 and 100%, remission rates between 42 and 100%, and recurrence rates between 3.0 and 63.2% depending on the experience of the surgeon and on the definition of remission. These rates have not improved significantly over the years. In experienced hands selective adenomectomy remains the least damaging and most effective treatment modality since it results in rapid clinical improvement if performed successfully. Therefore, it remains the treatment of choice. Patients not cured by surgery alone benefited from a combination of adjuvant treatment tailored to their specific needs using medications, radiation and/or adrenalectomy. In this fashion, we achieved normalization of cortisol levels in 79% and improvement in another 18% of the patients. We expect these rates to increase further once patients treated with radiotherapy begin to experience its full effect within the next few years.
我们评估了显微外科手术在库欣病(CD)治疗中的当前作用以及辅助治疗方式的疗效。原发性CD的标准治疗方法仍然是经蝶窦手术,对于持续存在高皮质醇血症的病例则进行辅助治疗。中度严重的病例采用放射治疗,而对于非常严重的病例则进行肾上腺切除术。在我们的原发性CD系列病例(1997年3月至2004年9月,平均观察期18.8个月)中,84.0%的病例在术中确诊为腺瘤。75.0%的患者实现了缓解,4.8%的患者出现复发。2.0%的病例发生了并发症,所有并发症均已解决,未导致永久性致残。在文献中,腺瘤的术中确诊率在59.1%至100%之间,缓解率在42%至100%之间,复发率在3.0%至63.2%之间,这取决于外科医生的经验和缓解的定义。这些比率多年来并未显著提高。在经验丰富的医生手中,选择性腺瘤切除术仍然是损害最小且最有效的治疗方式,因为如果手术成功,它会导致临床快速改善。因此,它仍然是首选的治疗方法。仅通过手术未治愈的患者受益于根据其特定需求定制的辅助治疗组合,使用药物、放疗和/或肾上腺切除术。通过这种方式,我们使79%的患者皮质醇水平恢复正常,另有18%的患者病情得到改善。我们预计,一旦接受放疗的患者在未来几年开始充分体验到放疗的效果,这些比率将进一步提高。