垂体细胞增生所致库欣病术后复发的诊断和处理

目的讨论垂体细胞增生所致库欣病经蝶术后复发的诊断和处理。方法根据临床症状,内分泌学检查和蝶鞍区MRI检查,5例病人被诊断为垂体ACTH腺瘤而行经蝶手术,术后病理为垂体细胞增生。术后1例症状无改善,4例症状改善3～11个月后复发皮质醇增高为降低皮质醇例行垂体放疗和肾上腺切除术,。2,3例行肾上腺切除术。在肾上腺切除的例中例症状32再次复发复查,MRI发现垂体腺瘤并再次经蝶手术,术后病理为垂体腺瘤。结果随诊1～5年,行二次经蝶手术的2例病人皮质醇降至正常行垂体放疗和肾上腺切除术者因垂体功能低下用强的松替代治疗结论当垂体细胞增生病人术后症状复发影像学。检查发现垂体肿瘤时应再次经蝶手术没有发现垂体腺瘤时可行肾上腺切除和垂体放疗。     

垂体Cushing病的经蝶手术治疗(附54例报告)

目的 回顾分析垂体Cushing病经蝶手术治疗的疗效,探讨其手术技巧及影响疗效的因素.方法 回顾2001年1月至2006年12月间经蝶手术的垂体Cushing病54例,其中微腺瘤39例,大腺瘤3例,核磁共振未见肿瘤征象者12例(通过地塞米松抑制试验和/或岩下窦采血证实为垂体Cushing病),术中行选择性腺瘤切除者38例,部分或半垂体切除者16例,术后病理证实为ACTH腺瘤者49例,垂体ACTH细胞增生者5例;术后缓解标准为术后第1 d或第2 d患者的血皮质醇≤5μg/L.结果 本组病人的缓解率75.9%,术前磁共振可显示肿瘤的患者缓解率(36/42,85.7%)高于未见肿瘤者(5/12,P<0.05),术中选择性腺瘤切除患者的缓解率(32/38,84.2%),亦高于其他(P<0.05),同样,术后病理证实为腺瘤的高于增生者(P<0.05);随访期内复发2例.结论 只要内分泌检查提示垂体Cushing病,即使磁共振未见肿瘤征象,也可行经蝶手术探查;从术前影像学表现、术中以及术后病理是否证实ACTH腺瘤可预测疗效.     

25例垂体ACTH腺瘤的临床分析

目的分析和探讨垂体促肾上腺皮质激素(ACTH)腺瘤的临床特点和治疗方法。方法回顾性分析从2004年1月至2006年3月在我院接受治疗的25例垂体ACTH腺瘤患者的临床特点、治疗经过及预后。结果垂体ACTH腺瘤患者的临床症状和肿瘤的大小密切相关,微腺瘤(直径≤10 mm)患者以Cushing综合症征为主要临床表现,而大腺瘤(直径>10 mm)患者则以压迫症状为主。有无Cushing综合征的表现和腺瘤的直径呈负相关(P<0.05)。本组25例垂体ACTH腺瘤中21例具有侵袭性。垂体ACTH腺瘤患者主要以育后女性为主。该病首次经蝶手术后近期疗效显著。结论垂体ACTH大腺瘤以压迫症状为主,微腺瘤则以Cushing综合征表现为主。该肿瘤大多数具有侵袭性倾向,育后女性是高危人群。经蝶显微手术是垂体ACTH腺瘤的首选治疗方法。     

老年性垂体腺瘤50例临床分析

目的探讨老年性垂体腺瘤的临床诊断和手术治疗特点。方法回顾性分析8年间50例老年垂体腺瘤患者的手术资料,其中11例采用经额手术,39例采用经蝶手术。结果肿瘤全切23例,次全切除15例,大部切除12例。结论对于有视力障碍的老年垂体腺瘤病例应采用手术治疗,尤其是经蝶入路切除垂体腺瘤,具有良好的耐受,高龄已经不再是禁忌证。     

儿童库欣病1例报告及文献复习

目的探讨儿童库欣病(CD)的临床特点及诊断、治疗方法。方法对南京大学医学院附属鼓楼医院神经外科2016年3月收治的1例儿童库欣病的临床表现、血液生化、影像学、病理学资料及显微外科手术方法和预后进行回顾性分析。结果患儿的肿瘤位于垂体下缘,与垂体分界尚清楚。一侧岩下窦与外周静脉血促肾上腺皮质激素(ACTH)的基础值之比≥2。MRI检查示,病变T_1WI表现为等信号或稍低信号,T_2WI表现为略高信号,增强后垂体下缘可见多发小结节样低信号影。术中见多个肿瘤样组织,暗红色,质韧。术后病理检查诊断为垂体腺瘤。免疫组化结果示,肿瘤细胞表达ACTH(散在+),PRL(-),FSH(部分+),TSH(-),P53(-),Ki167(小于1%+)。结论儿童垂体促肾上腺皮质激素微腺瘤有一定的特征性临床表现。增强MRI检查具有诊断参考作用,岩下窦采血检测ACTH有确诊意义。经蝶显微手术切除垂体腺瘤是治疗的最佳手段。     

儿童和青春期垂体促肾上腺皮质激素腺瘤的诊断和治疗

目的　讨论儿童和青春期垂体促肾上腺皮质激素 (ACTH)腺瘤的诊断和治疗。方法　回顾性分析经手术后病理证实为垂体腺瘤的 2 8例患者临床资料。结果　男 8例 ,女 2 0例 ,平均年龄 15 7岁。 2 6例有表现为典型的库欣病 (Cushing sdisease) ,10例有发育迟缓。均行经蝶窦显微外科垂体腺瘤切除术 ,2 6例行瘤周垂体切除。本组无死亡。随诊 1～ 10年 ,复发 5例 ,其中 3例再经蝶窦手术 ,2例行垂体放疗后 7年再次复发行肾上腺切除术 ,术后垂体功能低下 ,用强的松替代治疗。结论　儿童和青春期ACTH腺瘤大多数为垂体微腺瘤 ,发病年龄较小 ,可引起生长发育迟缓 ,经蝶窦显微外科垂体腺瘤切除术是最佳治疗方法 ,术中行瘤周垂体切除可提高疗效。对于临床症状复发 ,影像学检查有明显肿瘤复发者应再次经蝶窦手术治疗 ,而症状复发但影像学检查无明显肿瘤者 ,行蝶鞍区放疗或肾上腺切除手术。     

库欣病经蝶术后复发的诊断和治疗策略

目的 讨论库欣病经蝶术后复发的诊断和治疗。方法 男性9例，女性17例。年龄8～56岁，平均26．4岁。平均病程23．4个月。根据典型库欣综合征，内分泌学和影像学检查诊断为库欣病而行经蝶手术，术后病理垂体腺瘤18例，垂体细胞增生5例，病理阴性3例。术后症状复发，有16例再次行经蝶手术。结果 随诊1—12年，26例复发的库欣病经手术治疗19例皮质醇下降至正常，4例皮质醇低下需要补充激素，3例皮质醇仍高。结论 垂体促肾上腺皮质激素(ACTH)腺瘤复发者首先选择再次经蝶手术，病理为垂体细胞增生或病理阴性者不能排除垂体腺瘤的可能性，当症状复发时，如果MRI显示有垂体肿瘤应再次经蝶手术。垂体放疗和肾上腺切除术是辅助治疗方法。     

单鼻孔经蝶切除垂体微腺瘤的选择与技巧

目的探讨经蝶入路切除垂体微腺瘤的手术选择及技巧。方法回顾性分析经蝶入路显微手术治疗的30例垂体微腺瘤病人的临床资料,手术力争肿瘤包膜外切除,必要时适度电凝瘤床。免疫组化分型:泌乳素腺瘤8例,促肾上腺皮质激素(ACTH)腺瘤7例,生长激素腺瘤5例,促甲状腺激素(TSH)腺瘤1例,多分泌功能腺瘤5例,无功能腺瘤4例。结果手术全切肿瘤28例,次全切除2例(生长激素腺瘤和ACTH腺瘤各1例)。术后4个月,23例激素水平降至正常,2例下降大于50%,1例无明显变化。随访30例,时间6~78个月。临床症状均有不同程度改善。结论症状性无功能垂体微腺瘤可行经蝶手术治疗,功能性微腺瘤经蝶手术确切有效。术中精确定位与显露肿瘤以及熟练处理海绵间窦出血,是全切除垂体微腺瘤的重要前提。     

儿童库欣病的诊断和治疗

目的总结儿童库欣病的临床特点和治疗经验。方法回顾性分析27例年龄小于14岁儿童库欣病的临床资料。男15例,女12例,平均发病年龄10.7岁。术前影像学检查显示:微腺瘤20例,大腺瘤4例,未见异常3例。均行经蝶垂体微腺瘤切除加瘤周垂体大部切除术。结果肿瘤全切除26例,残留1例。术后病理证实垂体促肾上腺皮质激素(ACTH)腺瘤16例,垂体ACTH细胞增生3例,未发现腺瘤或增生7例。术后内分泌治愈率、缓解率和无效率分别是59.3%、7.4%和33.3%。平均随访30.4个月(6～120个月),复发4例(14.8%)。术后因复发或症状未缓解再次经蝶手术4例,垂体普通放疗5例,垂体伽玛刀放疗2例,肾上腺切除7例;出现Nelson综合征2例。结论经蝶垂体微腺瘤切除加瘤周垂体大部切除术是治疗儿童库欣病的首选和有效治疗方法,垂体放疗和肾上腺切除是二线治疗方法。     

垂体ACTH腺瘤经蝶术后复发的诊断与治疗

目的讨论复发性垂体促肾上腺皮质激素(ACTH)腺瘤的诊断和治疗.方法男性6例,女性12例.年龄8～43岁,平均25.7岁.平均病程29.5个月.均表现典型库欣综合征,诊断为垂体ACTH腺瘤行经蝶手术,术后病理为垂体腺瘤.术后皮质醇曾降至正常半年以上,再次出现库欣综合征,血和尿皮质醇增高,CT或MRI显示9例垂体腺瘤复发.12例采用再次经蝶手术,术中9例发现肿瘤,3例未发现肿瘤.6例未再行手术,其中4例行垂体放疗和肾上腺切除术,2例行肾上腺切除术.结果随诊1～12年,再次经蝶手术的治愈缓解率为58%(7/12).共有7例行垂体放疗加肾上腺切除术,4例行肾上腺切除术,术后皮质醇下降,有5例需用激素替代治疗.结论对于复发性垂体ACTH腺瘤首先应进行再次经蝶手术,如果术后皮质醇仍高,可行垂体放疗和/或肾上腺切除术.     

岩下窦静脉取血在疑难ACTH依赖性库欣综合征诊断中的应用

目的 探讨岩下窦静脉取血(IPSS)在疑难库欣综合征诊断中的价值.方法 对20例疑难库欣综合征行岩下窦和外周静脉取血,测定血ACTH浓度比,评价其对库欣病诊断的敏感性和特异性.结果 18例患者的岩下窦与外周静脉血ACTH的比值＞2,其中13例行经蝶窦垂体术后病理为垂体ACTH腺瘤;1例行鞍区γ-刀治疗、4例行生长抑素治疗后病情缓解.2例岩下窦与外周静脉血ACTH的比值＜2,其中1例为右肺类癌,另1例为垂体ACTH腺瘤.本组IPSS诊断库欣病的敏感性和特异性分别为93%和100%.结论 IPSS操作安全,并发症少,可作为疑难库欣综合征的重要鉴别方法.     

A case of non‐functioning pituitary adenoma with Cushing's syndrome upon recurrence

A 49‐year‐old woman presented with left visual disturbance. No signs of Cushing's disease were evident. Basal levels of serum cortisol and plasma adrenocorticotropic hormone (ACTH) were 16.8 μg/dL and 66.0 pg/mL, respectively. MRI demonstrated an irregularly shaped large pituitary tumor, and the patient then underwent transsphenoidal surgery. By light microscopy the tumor represented a chromophobic adenoma with a few of the adenoma cells showing immunoreactivity for ACTH. On the basis of clinical and light microscopic examinations, the diagnosis of silent corticotroph adenoma was made. Electron microscopy, however, demonstrated the honeycomb Golgi complex that has been reported as a typical finding of gonadotroph adenomas. MRI taken 7 months after the first operation revealed adenoma regrowth. Transcranial surgery was performed, and histology demonstrated a chromophobic pituitary adenoma with most cells immunopositive for ACTH. She was treated with gamma knife postoperatively. Three months later, MRI revealed remarkable shrinkage of the adenoma, but she developed typical signs and symptoms of Cushing's disease. Thus, the hormone immunostaining and biological activity of pituitary adenomas may change with time.     

Nelson综合征的显微外科治疗(附23例报告)

目的 分析和探讨Nelson综合征的病因,诊断标准和治疗方法。方法 对我科手术治疗的23例该病病例进行回顾性研究。临床特点包括肾上腺切除手术后皮肤粘膜色素沉着,血ACTH水平升高,影像学检查发现垂体腺瘤,21例经蝶窦手术,2例经额开颅,随访时间从半年至9年,结果本组23例Nelson综合征,占同期手术治疗的库欣病的7．7％,经蝶窦手术后皮肤色素沉着减轻,血ACTH水平下降,8例视力视野障碍术后均改善,治愈率56．5％,缓解率26．1％,结论 经蝶窦垂体ACTH腺瘤切除是预防和治疗Nelson综合征的首先方法,术后应定期随访。     

Cushing's disease in a young woman with anorexia nervosa: pathophysiological implications

This report describes a 17-year old student who was found to have Cushing's syndrome two years after she had developed anorexia nervosa (AN). The Cushing's syndrome was treated with bilateral resection of enlarged, hyperplastic, non-tumorous adrenal glands. The diagnosis was further confirmed four years later when, two to three years after new symptoms had appeared, an ACTH secreting pituitary adenoma (that is, Cushing's disease) was found on surgery. The possible mechanism for the development of Cushing's disease in a patient with prior anorexia nervosa, a sequence of events reported once previously, is discussed. It is suggested that increased hypothalamic-pituitary corticotroph stimulation in association with the anorexia nervosa, a now well-established endocrine phenomenon, activated an occult, inactive pituitary basophil adenoma in this patient, eventually resulting in autonomous pituitary overproduction of ACTH by the tumor.     

经蝶窦显微外科手术治疗541例库欣病

目的 探讨经蝶窦入路显微外科手术治疗库欣病的疗效。方法 总结北京协和医院神经外科1981年至2007年收治的541例库欣病的临床资料。结果CT和（或）MRI显示微腺瘤、大腺瘤、巨大腺瘤和未见异常的比例分别为66.2％( 358/541)、14.0％( 76/541)、0.6％( 3/541)和19.2％(104/541)。病理为垂体ACTH腺瘤、增生和既未见肿瘤又未见增生的比例分别为75.4％(408/541)、8.3％ (45/541)和16.3％ (88/541)。病理明确腺瘤、增生以及病理阴性者术后近期（半年内）治愈率分别是85.0％( 347/408)、24.4％ (11/45)和45.5％(40/88)。随访0.5 -25.0年（平均3.1年）,复发率13.3％(53/398),复发时间0.5 -16.5年（平均3.4年）。结论 经蝶窦选择性垂体腺瘤切除加瘤周垂体组织大部分切除是治疗库欣病安全有效的首选方法。对首次手术无效或复发性库欣病,可以再次经蝶窦手术或垂体放疗。     

单鼻孔经蝶入路切除垂体微腺瘤

目的探讨垂体微腺瘤的治疗理念和手术技术。方法报告15例手术,分析其经验和教训。结果临床诊断PRL腺瘤10例,GH腺瘤2例,ACTH腺瘤3例。手术全切肿瘤13例,大部切除2例,2例病理未证实肿瘤。男性PRL腺瘤疗效差,ACTH腺瘤手术切除常有一定难度。结论并非所有垂体微腺瘤都适于手术,宜慎重掌握手术指征。术中强调正确利用局部解剖关系,准确定位,合理止血,争取彻底切除。     

颈静脉孔异位垂体腺瘤的诊断及治疗(一例报告并文献复习)

目的 结合1例颈静脉孔异位垂体腺瘤患者的临床资料,探讨异位垂体腺瘤的发生机制、临床、病理特点及治疗方法.方法 回顾性分析1例颈静脉孔异位垂体腺瘤患者的临床资料.本患者临床表现为左耳耳鸣及间断性枕部疼痛,无内分泌功能紊乱症状,头颅MRI示左侧颈静脉孔区不规则椭圆形肿瘤影像,稍长T1、稍长T2信号,边界尚清楚,周边可见囊变,信号欠均匀,增强扫描病变不均匀强化.结果 行左侧乙状窦后入路,显微镜下全切除肿瘤,术后恢复顺利,病理回报ACTH型垂体腺瘤.术后化验ACTH值为23.7 pg/ml,其他内分泌指标均正常.结论 异位于颈静脉孔的垂体腺瘤少见,术后病理是诊断的主要依据,手术治疗效果好,如有内分泌功能紊乱症状在给予手术治疗的同时,可合理选择放疗和药物治疗.     

Diagnostic value of super-selective bilateral cavernous sinus sampling with hypothalamic stimulating hormone loading in patients with ACTH-producing pituitary adenoma

AIMS: Early diagnosis and early treatment by transsphenoidal surgery is desirable for ACTH-producing pituitary microadenoma, but accurate localization of the functional lesion is not always possible before surgery because magnetic resonance (MR) imaging may provide false negative and/or positive findings. The diagnostic value of super-selective bilateral cavernous sinus sampling with the administration of corticotropin-releasing hormone (CRH) was assessed in patients with functioning ACTH-producing pituitary adenoma. METHODS: Fifteen patients with pituitary adenoma (14 with microadenoma) aged from 23 to 74 years (mean 46.7 years) underwent cavernous sinus sampling with or without the CRH loading test and subsequent transsphenoidal surgery in our institute from October 1997 through to November 2002. MR imaging including dynamic scan failed to detect the adenomatous lesion in all patients. To eliminate the bias due to uneven blood flow in the cavernous sinuses and the multi-hormonal response to CRH administration, the ACTH/FSH ratios were evaluated. The inter-cavernous gradient (ICG) was calculated as the higher/lower ACTH venous blood levels in the right and left cavernous sinuses with or without CRH loading. The adjusted ICG was calculated using the ACTH/FSH ratios. The results were compared with the surgical findings. An ICG of 1.4 or greater was considered to indicate the localization of the responsible lesion. RESULTS: Transsphenoidal surgery revealed the functioning lesion on the right in five cases, the left in six, the midline in three and the bilateral lateral wings (double adenoma) in one. Adjusted ICG with CRH loading had a localization accuracy of 93.3% (14/15), which was significantly higher than that of 73.3% (11/15) using ICG without hypothalamic stimulating hormone loading (p = 0.0402). CONCLUSIONS: Super-selective cavernous sinus sampling with hypothalamic stimulating hormone administration can provide accurate localization of the responsible lesion in patients with ACTH-producing pituitary adenoma.     

Silent mixed corticotroph and somatotroph macroadenomas presenting with pituitary apoplexy

We discuss three unique cases of pituitary macroadenoma presenting with pituitary hemorrhage but without typical endocrine symptomatology. Immunohistochemical analysis indicated positive reactivity for adrenocorticotropic hormone (ACTH) and growth hormone (GH), and in situ hybridization indicated the expression of proopiomelanocortin (POMC) and GH mRNA. We designated these cases silent mixed corticotroph and somatotroph adenoma. Patient 1 was a 30-year-old man, patient 2 was a 29-year-old woman, and patient 3 was a 59-year-old woman. All patients presented with a headache of sudden onset and visual disturbance. The patients did not exhibit typical Cushing's or acromegalic features. Serum ACTH level was remarkably elevated in patient 1, and slightly elevated in patients 2 and 3. In all patients, serum GH levels were within normal range and magnetic resonance imaging revealed an intra- and suprasellar mass with pituitary hemorrhage. Transnasal pituitary surgery in the three patients disclosed a pituitary adenoma producing ACTH and GH. In patient 2, the residual adenoma reappeared along with an intratumoral hemorrhage, and was resected by secondary transnasal surgery. Silent mixed corticotroph and somatotroph adenomas are characterized by the following: no endocrine symptoms; presentation dominated by mass effect symptoms; macroadenoma presenting with acute pituitary hemorrhage; and production of both ACTH and GH.