1Department of Neurosurgery.
2Department of Pathology, and.
J Neurosurg. 2018 Nov 1;129(5):1260-1267. doi: 10.3171/2017.5.JNS162901.
OBJECTIVEThe authors report their single-institution experience with the pathological findings, rates of remission, and complications in patients with presumed Cushing's disease (CD) who underwent a two-thirds pituitary gland resection when no adenoma was identified at the time of transsphenoidal surgery (TSS). The authors also review the literature on patients with CD, negative surgical exploration, and histological findings.METHODSThis study is a retrospective analysis of cases found in neurosurgery and pathology department databases between 1989 and 2011. In all cases, patients had been operated on by the same neurosurgeon (K.O.L.). Twenty-two (13.6%) of 161 patients who underwent TSS for CD had no adenoma identified intraoperatively after systematic exploration of the entire gland; these patients all underwent a two-thirds pituitary gland resection. A chart review was performed to assess treatment data points as well as clinical and biochemical remission status.RESULTSOf the 22 patients who underwent two-thirds gland resection, 6 (27.3%) ultimately had lesions found on final pathology. All 6 patients were found to have a distinct adrenocorticotropic hormone (ACTH) cell adenoma. Sixteen (72.7%) of the patients had no tumor identified, with 3 of these patients suspected of having ACTH cell hyperplasia. The follow-up duration for the entire group was between 14 and 315 months (mean 98.9 months, median 77 months). Remission rates were 100% (6/6 patients) for the ACTH cell adenoma group and 75% (12/16) for the group without adenoma. Overall, 18 (81.8%) of the 22 patients had no evidence of hypercortisolism at last follow-up, and 4 patients (18%) had persistent hypercortisolism, defined as a postoperative cortisol level > 5 μg/dl. Of these 4 patients, 1 was suspected of harboring a cavernous sinus adenoma, 2 were found to have lung tumors secreting ACTH, and 1 remained with an undiagnosed etiology. Rates of postoperative complications were low.CONCLUSIONSThe diagnosis and treatment of CD can be challenging for neurosurgeons, endocrinologists, and pathologists alike. Failure to find a discrete adenoma at the time of surgery occurs in at least 10%-15% of cases, even in experienced centers. The current literature provides little guidance regarding rational intraoperative approaches in such cases. The authors' experience with 161 patients with CD, when no intraoperative tumor was localized, demonstrates the utility of a two-thirds pituitary gland resection with a novel and effective surgical strategy, as suggested by a high initial remission rate and a low operative morbidity.
报告作者在一家机构的经验，这些患者被诊断为库欣病（CD），在经蝶窦手术（TSS）时未发现腺瘤，术后行三分之二垂体切除术，研究其病理结果、缓解率和并发症。作者还回顾了关于 CD 患者、阴性手术探查和组织学发现的文献。
本研究是对 1989 年至 2011 年神经外科和病理科数据库中病例的回顾性分析。所有患者均由同一位神经外科医生（K.O.L.）进行手术。22 例（161 例中的 13.6%）CD 患者在系统探查整个腺体后术中未发现腺瘤，均行三分之二垂体切除术。进行图表审查以评估治疗数据点以及临床和生化缓解状态。
在接受三分之二腺体切除术的 22 例患者中，6 例（27.3%）最终在最终病理中发现病变。所有 6 例均发现有明显的促肾上腺皮质激素（ACTH）细胞腺瘤。16 例（72.7%）患者未发现肿瘤，其中 3 例疑为 ACTH 细胞增生。整个组的随访时间为 14 至 315 个月（平均 98.9 个月，中位数 77 个月）。ACTH 细胞腺瘤组的缓解率为 100%（6/6 例），无腺瘤组的缓解率为 75%（12/16 例）。总体而言，22 例患者中有 18 例（81.8%）在末次随访时无皮质醇增多症证据，4 例（18%）皮质醇增多症持续存在，定义为术后皮质醇水平>5μg/dl。这 4 例患者中，1 例疑为海绵窦腺瘤，2 例发现有分泌 ACTH 的肺部肿瘤，1 例仍未明确病因。术后并发症发生率低。
神经外科医生、内分泌学家和病理学家在诊断和治疗 CD 时都可能面临挑战。即使在经验丰富的中心，手术时也至少有 10%-15%的病例未能发现离散的腺瘤。目前的文献几乎没有提供关于此类病例合理的术中方法的指导。作者对 161 例 CD 患者的经验表明，当术中未定位到肿瘤时，采用三分之二垂体切除术的新有效手术策略是有效的，这可以从较高的初始缓解率和较低的手术发病率得到证明。
