Department of Neurosurgery, University of Erlangen-Nürnberg, Erlangen, Germany.
Neuroendocrinology. 2010;92 Suppl 1:102-6. doi: 10.1159/000314223. Epub 2010 Sep 10.
In this article, the present status of neurosurgical operations for Cushing's disease is briefly reviewed. Transsphenoidal surgery is considered the treatment of choice in most patients with Cushing's disease once the diagnosis has been established. In a considerable proportion of patients, even sophisticated imaging does not directly depict the tiny microadenoma. The search for the tumor is technically difficult, particularly when the sella turcica is small, the dura vascularized and the sphenoid sinus poorly pneumatized. Thus, even in expert hands, microadenomas cannot always be identified intraoperatively. Usually, a selective adenomectomy is attempted, preserving pituitary functions. There is a huge variation of surgical outcomes reported. As an estimate, a remission rate of some 75% can be expected 5 years after surgery. Almost all data available to date derive from microsurgical operations. Unfortunately, even in patients who initially remit, recurrences may occur. Low postoperative serum cortisol levels and a long-lasting adrenocortical insufficiency seem to be factors associated with a favorable long-term outcome. When no distinct microadenoma can be identified intraoperatively, partial or even total hypophysectomy has been suggested. However, the outcome of these procedures is less favorable than with selective resections of distinct adenomas. Less than 10% of pituitary adenomas associated with Cushing's disease are macroadenomas. These also bear a less favorable outcome than microadenomas. Only for selected patients with mainly extrasellar tumor localizations are craniotomies recommended. A close cooperation with the endocrinologist is mandatory for a neurosurgeon operating on patients with Cushing's disease, namely for the pre- and perioperative care and for long-term follow-up.
本文简要回顾了神经外科治疗库欣病的现状。一旦确诊库欣病,经蝶窦手术被认为是大多数患者的首选治疗方法。在相当一部分患者中,即使是复杂的影像学检查也不能直接显示微小的微腺瘤。肿瘤的寻找在技术上具有挑战性,尤其是当蝶鞍较小、硬脑膜血管化和蝶窦充气不良时。因此,即使是在经验丰富的手中,也不能总是在术中识别出微腺瘤。通常,会尝试进行选择性腺瘤切除术,以保留垂体功能。据报道,手术结果存在巨大差异。估计术后 5 年的缓解率约为 75%。迄今为止,几乎所有可用的数据都来自微创手术。不幸的是,即使在最初缓解的患者中,也可能会复发。术后血清皮质醇水平较低和长期肾上腺皮质功能不全似乎与良好的长期预后相关。如果术中无法明确识别出微腺瘤,则建议进行部分或全部垂体切除术。然而,这些手术的结果不如明显腺瘤的选择性切除。与库欣病相关的垂体腺瘤中,不到 10%为大腺瘤。这些腺瘤的预后也比微腺瘤差。只有对于主要位于鞍外的特定患者,才推荐进行开颅手术。神经外科医生在为库欣病患者进行手术时,必须与内分泌科医生密切合作,包括术前和围手术期护理以及长期随访。