伴肢端肥大症垂体腺瘤患者的免疫组化研究

作者应用免疫组织化学方法测定了30例伴有肢端肥大症垂体腺瘤患者的HGH、PRL及ACTH激素抗血清染色,结果27例HGH阳性,其中HGH和PRL混合阳性8例,HGH、PRL和ACTH混合阳性2例。本文对垂俸腺癌组织病理与激素组织定位和临床症状的关系进行了探讨,阐明了免疫组化对于垂体腺瘤功能分类的意义。     

肢端肥大症的并发症

肢端肥大症可合并多种并发症,本文简要介绍肢端肥大症合并心血管系统疾病、糖代谢异常、骨关节病变等并发症,以及经手术、放疗等治疗后这些并发症的转归。     

肢端肥大症继发糖尿病酮症酸中毒临床分析

糖代谢紊乱是肢端肥大症的一个常见的临床特征。由于垂体分泌过量的生长激素(GH)可造成显著的胰岛素抵抗,继而引发糖代谢紊乱。北京协和医院报道的一组肢端肥大症550例[1],做葡萄糖耐量试验(OGTT),糖尿病的发生率为18.5%,糖耐量异常(IGT)为40%。Arya等[2]资料中56%的肢端肥大症继发糖尿病。但由肢端肥大症引起的糖尿病酮症酸中毒(DKA)则罕见,国外仅有个案报道[3~5]。由于肢端肥大症起病隐匿、进展缓慢,因而常常不能及时就诊明确诊断,有时以DKA为首发表现[6],更易造成误诊。现将我们收治的4例患者,其中3例以DKA为首发表现,现报道如下…     

肢端肥大症11例并发症临床分析

目的研究肢端肥大症并发症发生情况。方法选取浙江大学附属第一医院2000~2004年收治的11例肢端肥大症患者进行回顾性分析。结果肢端肥大症并发症发生率分别为:糖尿病72.7%、脂代谢紊乱45.5%、心血管疾病45.5%、甲状腺疾病18.2%。结论有效地控制肢端肥大症原发病可减少其并发症的发生或延缓其并发症的发展。     

以糖尿病为首发表现的肢端肥大症两例

病例1:患者,男性,59岁.因"口干、多饮、多食伴消瘦半个月余"入住我科.测血糖18 mmol/L,考虑初发糖尿病.家族无类似病史.体格检查:体温37℃,呼吸20次/分,脉搏80次/分,血压110/70 mm Hg(1 mm Hg =0.133 kPa),体重45 kg,身高172 cm,体质量指数23.83 kg/m2,肢端肥大面容,体型中等,余心、肺、腹无异常.实验室检查:空腹血糖(FBG) 12.4mmol/L,餐后2小时血糖(2hBG) 19.3 mmol/L,空腹C肽0.48ng/ml(0.81 ～ 3.85ng/ml),餐后2小时C肽0.52 ng/ml,糖化血红蛋白比例(HbA1C) 12.42％,抗胰岛素抗体、抗胰岛细胞抗体、抗谷氨酸脱羧酶抗体均阴性,高糖抑制试验:生长激素(GH)(0'):25.9 μg/l,生长激素(30'):15.4 μg/l,生长激素(60'):18.3 μg/L,生长激素(120'):30.7 μg/L,类胰岛素样生长因子-1(IGF-1)555 μg/l,肝肾功能电解质正常,垂体前叶其他激素(甲状腺功能、促肾上腺皮质激素、皮质醇、睾酮、黄体生成素及卵泡生成素)正常.     

生长介素对肢端肥大症患者垂体瘤生长激素分泌影响的体外研究

作者在8例肢端肥大症体外培养的垂体GH瘤细胞上探讨IGF-1对GH分泌的反馈调节作用。10~(-7)mol IGF-1使3例垂体GH瘤细胞GH基础分泌抑制到对照的44.6％～52.4％(P<0.05),1例GH分泌增加134.9％(P<0.05),4例GH基础分泌没有明显改变,表明体外培养的垂体GH瘤有一半以上对IGF-1 3小的·的急性作用失去正常的GH分泌抑制反应。3例对IGF-1失去GH分泌抑制反应的垂体GH瘤细胞同时伴有GH对GHRH_(1-44)及(或)生长抑素激动剂SMS_(201-995)反应消失,提示部分垂体GH瘤细胞的IGF-1和某些下丘脑激素受体或受体后有异常。     

正常成人及肢端肥大症患者的生长激素谱测定

对10例正常成人及4例肢端肥大症(肢大)患者进行生长激素(GH)谱测定,正常人GH分泌型式为长期分泌静止,间以脉冲分泌,在第1次深睡后有GH分泌大峰;女性GH分泌波动较男性明显。肢大患者均无夜间GH分泌大峰。2例测24小时GH谱的肢大患者中,1例血糖水平正常男性患者GH分泌峰数增多,另1例并有高血糖女性患者GH分泌频率明显减少。测定结果表明垂体GH分泌瘤的功能虽系自主性,但仍部分受下丘脑释放激素的调控。     

肢端肥大症的药物治疗进展

垂体肿瘤分泌过多生长激素（GH）是导致肢端肥大症的最主要的原因，其治疗手段包括手术、药物和放疗。对于垂体微腺瘤者，外科手术仍然是首选的治疗方式，药物仅起辅助作用；对垂体大腺瘤而言，药物治疗，尤其是生长抑素类似和新近开发的GH受体拮抗剂的应用具有更加重要的地位。     

如何看待肢端肥大症患者手术后生长激素的轻度"异常"

生长激素和胰岛素样生长因子I(IGF I)测定是肢端肥大症诊断和复发监测的重要手段。结合75 g口服葡萄糖耐量试验(OGTT) ,升高的血糖可以抑制垂体分泌生长激素,在正常人,OGTT过程中,生长激素的最低值可被抑制到1μg/L以下(译者注:据北京协和医院资料,正常人在5 0～10 0 gOGTT试验中生长激素最低水平为0 .2～0 .3 μg/L ,一般认为2 μg/L以下属正常)。Freda等报道正常人OGTT中生长激素水平低于0 .14 μg/L。结合OGTT进行的生长激素测定有助于对自主性生长激素分泌肿瘤的鉴别诊断。Freda等观察了76例手术治疗后的肢端肥大症患者,这…     

垂体生长激素瘤合并甲状腺高功能腺瘤一例报告并文献复习

生长激素瘤合并甲状腺高功能腺瘤临床较为罕见,本文报道1例垂体生长激素瘤合并甲状腺高功能腺瘤患者的临床特点、治疗、转归,并对两者关系的近期国内外研究进行复习.     

Gallbladder villous adenoma in a patient with acromegaly： A case report

Villous adenomas are benign epithelial lesions with malignant potential that can occur in any part of the gastrointestinal tract. We present a case of a middle age woman with acromegaly who was investigated for nonspecifi c gastrointestinal complaints. Ultrasonography and subsequent endosonography diagnosed a large (4.5 cm), hyperechoic, sessile polyp with numerous pedicles. An open cholecystectomy was performed and revealed a villous adenoma with several foci of carcinoma in situ. Detailed investigations showed no other tumors of the gastrointestinal tract. After fi ve years of follow-up, the patient reports no complaints, and the results of laboratory testing and imaging studies are within the normal range.     

Modification of hormonal secretion in clinically silent pituitary adenomas

Background Silent pituitary adenomas are a subtype of adenomas characterized by positive immunoreactivity for one or more hormones classically secreted by normal pituitary cells but without clinical expression, although in some occasions enhanced or changed secretory activity can develop over time. Silent corticotroph adenomas are the classical example of this phenomenon. Patients and Methods A series of about 500 pituitary adenomas seen over a period of 20 years were screened for modification in hormonal secretion. Biochemical and immunohistochemical data were reviewed. Results Two cases were retrieved, one silent somatotroph adenoma and one thyrotroph adenoma, both without specific clinical features or biochemical abnormalities, which presented 20 years after initial surgery with evidence of acromegaly and hyperthyroidism, respectively. While the acromegaly was controlled by a combination of somatostatin analogs and growth hormone (GH) receptor antagonist therapy, neurosurgery was necessary to manage the thyrotroph adenoma. Immunohistochemical examination demonstrated an increase in the number of thyroid stimulating hormone (TSH)-immunoreactive cells compared to the first tissue. Apparently, the mechanisms responsible for the secretory modifications are different, being a change in secretory capacity in the silent somatotroph adenoma and a quantitative change in the silent thyrotroph adenoma. Conclusions These two cases, one somatotroph and one thyrotroph adenoma, are an illustration that clinically silent pituitary adenomas may in rare circumstances evolve over time and become active, as previously demonstrated in silent corticotroph adenomas.     

手术治疗121例老年垂体腺瘤临床分析

目的 探讨老年垂体腺瘤的临床特点和手术治疗意义.方法 回顾性分析了121例60岁及以上垂体腺瘤的临床资料.结果 手术主要采用经鼻蝶窦入路,肿瘤全切除及次全切除91例,大部切除25例,部分切除5例.住院期间视力好转80例,占88.9％.随访MRI72例,肿瘤无复发或静止状态67例,复发需再次手术5例.术后生活自理率80％.结论 多数老年垂体腺瘤初期的临床表现隐匿,发现时往往肿瘤大、压迫视神经,尽早鞍区MRI检查可明确诊断.手术切除肿瘤对老年垂体瘤有效、安全,尤其是经鼻蝶入路.     

124例伴血催乳素增高的生长激素分泌性垂体腺瘤临床分析

目的探讨伴血催乳素(PRL)增高的生长激素(GH)分泌性垂体腺瘤的临床特点。方法采用回顾性病例研究方法,对北京协和医院1984年至2004年收治的124例伴血PRL增高的GH分泌性垂体腺瘤患者进行临床和激素水平分析,其中87例随诊1年以上。结果124例患者中女性88例,男性36例,男性病程(8.0±7.3)年长于女性(5.5±4.3)年(P<0.01)。男性和女性患者性腺功能低减者分别占71.6%和69.4%。87例随访患者随访(5.5±4.3)年,其中垂体微腺瘤3例,非侵袭性大腺瘤28例,侵袭性大腺瘤56例。肿瘤免疫组化染色呈单纯GH( )占19.6%,其余多呈多激素染色阳性。血PRL水平与肿瘤体积呈正相关(r=0.261,P<0.05)。鞍上型肿瘤中,单纯GH腺瘤的血PRL值(58.7±1.5)μg/L,明显低于多激素染色腺瘤者的(90.3±2.4)μg/L(P<0.05)。87例随访患者的治愈率为27.6%。结论伴血PRL增高的GH分泌性垂体腺瘤好发于女性,性腺功能低减发生率高,治愈率低,血PRL的水平和肿瘤体积有关,是GH分泌性垂体腺瘤中有独特的临床特点及转归的一种特殊临床亚型。     

A comparative study of functioning and non-functioning pituitary adenomas

Pituitary tumors commonly cause visual impairment and the degree of impairment can depend on the size, location, and type of the tumor. However, no studies have been made regarding the differences caused by functioning pituitary adenoma (FPA) and non-functioning pituitary adenoma (NFPA). We aimed to investigate the relationship between clinical characteristics and visual impairment in patients with FPA and NFPA.This case series study included 73 pituitary adenoma patients. All patients underwent ophthalmic evaluations, and we retrospectively reviewed their medical records. Tumor types were confirmed by histological analysis, and the tumor volume was calculated. Magnetic resonance imaging was used to determine the tumor diameter. The observation indices of the two groups were compared. The correlation between the visual field and tumor volume was analysed using scatter plots.We enrolled 30 patients in the FPA group and 43 in the NFPA group. The first symptoms presented in the eyes in 23% of FPA patients and 41.9% of NFPA patients. The best-corrected visual acuity of the FPA group was better than that of the NFPA group, and 34 (56.7%) and 73 (84.9%) eyes in these groups had visual field defects, respectively. The visual field defects of the FPA patients were lighter than those of the NFPA patients. Except for the anteroposterior diameter, there were no differences in the other parameters of tumor diameter between the groups. The tumor volume of the FPA group was smaller than that of the NFPA group. The tumor size was positively correlated with the mean deviation and negatively correlated with the mean sensitivity in both groups.There was a longer delay between the onset of signs and symptoms and treatment in the FPA group than in the NFPA group. Future studies should focus on visual field defects caused by FPA and NFPA.     

让垂体瘤的治疗更趋完善

垂体瘤患者术后进行垂体功能的评估可以及时地对垂体功能减退者进行合适的替代治疗.但我们当前的临床现实是能够接受恰当的术后评估的患者还不到50％.只有加强内分泌科与神经外科医师的技术互补协作以及三级医院医师和基层医院医师的密切联系,提供以患者为中心的治疗才能弥补当前垂体瘤治疗存在的缺陷.     

Thrombotic thrombocytopenic purpura associated with a prolactin-producing pituitary adenoma

We report here the case of a 44-year-old woman with thrombocytopenia, anemia, convulsions, hyperprolactinemia, and galactorrhea. The patient died of cardiac failure. Autopsy revealed PAS-positive and von Willebrand factor-positive microthrombi in the arterioles and capillaries of many organs, mainly in the heart and brain, confirming the clinical diagnosis of thrombotic thrombocytopenic purpura. In the pituitary, a prolactin-producing adenoma was identified. To our knowledge, thrombotic thrombocytopenic purpura accompanied by a prolactin-secreting pituitary adenoma has not yet been described. The question of whether the association between the vascular changes and the pituitary adenoma is incidental or causal cannot be answered. Further studies are required to determine whether prolactin released from the pituitary tumor in excess played a role in the formation of microthrombi causing multiple organ failure and the demise of the patient.     

垂体促甲状腺激素腺瘤的临床特征和术前短效奥曲肽治疗的临床疗效

目的归纳分泌促甲状腺激素(TSH)垂体腺瘤(TSH腺瘤)的临床特征,评价术前短效奥曲肽治疗的临床疗效。方法回顾性分析首都医科大学附属北京天坛医院2008年1月至2018年1月明确诊断的垂体TSH腺瘤患者共40例。采集一般资料、实验室及影像学检查等临床资料;评价患者术前奥曲肽治疗的临床疗效。结果40例患者(男:女=24∶16),发病年龄(30.5±5.1)岁,大腺瘤35例(87.5%);最常见症状为甲状腺高代谢症候群,共32例(80.0%),其次是头疼、头晕,视力、视野受损和性腺功能减退。30例(75%)患者奥曲肽治疗3~5d甲状腺功能即恢复正常,总有效率90.0%;有效累计剂次超过10次的患者其治疗前游离甲状腺素(FT4)水平明显高于10次以下者。结论甲状腺高代谢症候群、垂体占位效应是垂体TSH腺瘤最常见的临床表现,术前奥曲肽治疗可有效控制甲状腺功能亢进,治疗前FT4水平是影响奥曲肽疗效的重要因素。     

Current perspectives on recurrent pituitary adenoma: The role and timing of surgery vs adjuvant treatment

The clinical course of pituitary adenoma can be highly variable. Aggressive pituitary tumours may require multimodal therapy with multiple operations. Even standard pituitary adenomas exhibit relatively high long-term recurrence rates and delayed intervention is often required. The indications for revision surgery in the endoscopic era are expanding for both functioning and nonfunctioning tumours, including access to the cavernous sinus and intracranial compartments. Although revision surgery can be challenging, it has been demonstrated to be both safe and effective. The question of the use of early radiotherapy in pituitary adenoma remains controversial. Our increasing understanding of pituitary tumour biology facilitates individualized treatment and surveillance protocols, with early intervention in high-risk adenoma subtypes. In this review, we discuss the treatment options for recurring pituitary tumours and focus on the role of revision surgery.