垂体前叶功能减退症117例临床分析

目的 探讨垂体前叶功能减退症临床变化特征.方法 回顾性分析本院1994年至2010年住院及部分门诊117例患者的资料.结果 男性占60.68％,女性39.32％;诊断时年龄13 ～79岁,40岁以上占79.12％;鞍区肿瘤57例(40.17％),发生在手术和(或)放疗后55例(占鞍区肿瘤的96.49％),经额入路手术患者35例(66.04％),经蝶入路手术患者18例(33.96％);首发症状以ACTH缺乏致乏力、厌食、恶心呕吐的低钠血症108例(92.31％)最常见;伴中枢性尿崩症23例(19.66％),伴冠心病82例(70.09％).结论 本症由于致病因素的变化,发病人群、首发症状及受累激素均已发生很大变化.患者男性多于女性,发病年龄偏高,病因以垂体瘤为主.     

746例经手术治疗的垂体腺瘤患者临床资料分析

目的 分析不同类型垂体腺瘤的临床、病理、影像学特点,探讨垂体瘤诊断相关问题.方法 回顾性研究746例垂体瘤患者的临床资料,包括临床表现、病理学、影像学特点.结果 不同类型垂体瘤患者有其各自的临床特征,但以不同程度的压迫症状为主,或伴有激素水平异常的症状.催乳素>121.28μg/L时多考虑催乳素腺瘤(灵敏度79.9%,特异度97.2%).不同类型垂体腺瘤的体积有显著性差异(P<0.01),肿瘤侵袭性和肿瘤大小有显著正相关性(P<0.01).结论 不同类型垂体瘤特点各异,需要针对不同类型的垂体瘤患者进行个体化诊断,治疗和预后评估.     

内镜下经鼻蝶入路二次手术治疗复发垂体瘤245例

目的探讨内镜下经鼻蝶入路二次手术治疗复发垂体瘤患者的临床有效性及安全性,并总结手术操作技巧。方法选取2008-03~2014-02在兰州市第一人民医院脑外科住院的垂体瘤复发患者245例,患者初次垂体瘤手术方式为经蝶入路或开颅手术,后给予其二次内镜下经鼻蝶手术治疗,观察患者瘤体切除程度、术后视力及内分泌变化及并发症的发生情况,分析二次经蝶入路手术治疗垂体瘤的有效性及安全性。结果内镜下经鼻蝶入路二次手术治疗垂体瘤全切加次全切除率达80.38%,无严重并发症发生,无死亡病例。结论内镜下经鼻蝶入路二次手术治疗复发垂体瘤安全有效,值得临床推广应用。     

垂体腺瘤患者视功能障碍临床研究

目的 探讨垂体腺瘤视功能障碍的发生机制及经鼻蝶入路垂体瘤切除术后视功能恢复的规律.方法 对180例经鼻蝶人路手术的垂体腺瘤患者按肿瘤病理分型、瘤体大小、性别、年龄分类,分别测定术前及术后的视力、视野、眼底情况.Logistic多因素分析垂体瘤患者视功能障碍的预测因素.结果 180例患者中,有视力障碍者占68.89%,巨大腺瘤有视功能下降92.59%,大腺瘤有视功能障碍占66.18%,微腺瘤有视功能障碍52.94%,其中7例为视野改变.手术3个月后视力好转101例(81.45%),无改变30例(24.19%),恶化3例(2.42%).眼底改变大多数为原发性视神经萎缩,伴卒中患者出现视乳头水肿.结论 视功能障碍与肿瘤高度、前后径及年龄相关.     

内镜辅助下经鼻蝶入路手术治疗垂体瘤的临床效果观察

目的探讨内镜辅助下经鼻蝶入路手术治疗垂体瘤的临床效果。方法将该院2017-05～2018-05收治的92例垂体瘤患者,根据手术方法不同分为对照组和研究组,对照组行经鼻蝶显微镜垂体瘤切除术治疗,研究组行内镜辅助下经鼻蝶入路手术治疗。对比两组患者的临床疗效及两组患者内分泌激素水平及术后并发症发生情况。结果研究组的临床疗效优于对照组(P0.01),同时研究组的术后并发症总发生率显著低于对照组(P0.01);治疗后研究组血管紧张素、肾素、去甲肾上腺素等内分泌激素水平低于对照组(P0.01)。结论内镜辅助下经鼻蝶入路手术治疗垂体瘤患者的效果更佳,患者内分泌激素水平的影响相对较低,治疗有效率高,不良反应少,值得进一步推广。     

催乳素瘤治疗的研究进展

催乳素瘤是最常见的一种垂体瘤。药物治疗可使超过90%的患者血催乳素水平正常,但需要长期服药。溴隐亭、卡麦角林和喹高利特等都有降低血催乳素水平和缩小瘤体的作用。卡麦角林被认为比溴隐亭更有效且耐受性好。因已证明在孕期溴隐亭具有安全性而被推荐用于孕期治疗。手术一般用于药物治疗无效或不耐受者。放射治疗仅用于手术不能切除肿瘤及术后肿瘤复发或药物治疗失败的患者。     

垂体瘤术后并发垂体前叶功能减退致甲状腺功能减退性心脏病一例

正垂体瘤是常见的颅脑肿瘤,约占颅内肿瘤的5%~20%。根据能否合成和分泌有生物活性的激素,临床上将垂体瘤分为功能性瘤(包括生长激素瘤、泌乳素瘤、促肾上腺皮质激素瘤、促甲状腺激素瘤)和无功能瘤。除泌乳素瘤外,手术(经颅或者经蝶窦入路)是首选的治疗方式。据文献报道,垂体微腺瘤(直径1 cm)手术切除治疗的成功率是70%左右,而大腺瘤则≤30%~([1])。因此,对于大腺瘤,术后定向放疗,如伽玛刀是一种较为安全的辅助治疗方式。术后伽玛刀放疗     

垂体瘤61例临床诊治分析

目的总结垂体瘤的诊治经验。方法回顾性分析南京军区南京总医院2001—2006年61例垂体瘤患者的临床资料。评价其发生率、临床表现、内科及外科治疗方法的疗效。结果大腺瘤共48例,手术以经蝶窦术式为主。内科治疗以溴隐停为代表的多巴胺2受体激动药为首选。结论垂体瘤中催乳素瘤和生长激素瘤发病率较高,分别占23%、20%。大腺瘤的治疗主要采取手术治疗。     

胰腺神经内分泌肿瘤:临床病例回顾性分析

目的回顾性分析胰腺神经内分泌肿瘤的临床病例资料,加强对该病的认识和诊治。方法收集南京军区南京总医院1999年1月-2012年12月的37例胰腺神经内分泌肿瘤住院患者的临床症状、影像学检查、病理、手术治疗、随访等资料进行分析。结果本组患者中,33例(89.2%)为非功能性肿瘤,4例(10.8%)为功能性肿瘤。非功能性肿瘤以腹痛、腹胀、腹泻、黄疸为主要表现,8例无任何症状;功能性肿瘤以低血糖反应为主要表现。病灶的检出率B超为80%,CT、MRI以及PET/CT均为100%,病变部位以胰头部最常见,瘤体平均直径为5.7 cm(1.4~20 cm)。病理学分类以神经内分泌癌最常见,其次为胰岛细胞瘤,免疫组化CgA、Syn、NSE、CKpan的阳性率分别为66.7%、92.9%、87.5%、73.3%。有15例患者发生肿瘤转移,以肝脏(6例)和淋巴结(5例)转移常见。27例患者行手术治疗,手术相关并发症发生率为29.6%(8/27),无手术相关的死亡病例。术后随访病例34例,其中8例复发,3例死亡。结论胰腺神经内分泌肿瘤的临床症状无明显特异性,影像学检查有助于早期诊断肿瘤、发现转移灶及术前评估,手术治疗有助于延长患者生存期,术后密切随访有利于早期发现和处理复发病灶。     

单鼻孔经蝶入路切除垂体腺瘤术后并发症的发生原因及与预防措施

垂体瘤是颅内常见的肿瘤,可引起多种内分泌症状及多脏器、器官损害。2010年5月-2011年4月,我们共对39例垂体腺瘤患者行单鼻孔经蝶入路手术,其中19例术后出现不同类型并发症。现分析并发症发生原因并提出预防对策。     

Pituitary Apoplexy During Therapy with Cabergoline in an Adolescent Male with Prolactin-Secreting Macroadenoma

Pituitary adenomas are rare in young patients. Prolactinomas are the most common type of pituitary adenomas in children older than 12 years, occurring more often in girls, at a 4.5:1 female-to-male ratio. The clinical presentation may vary according to the age and sex of the patient. Pituitary apoplexy is a rare life-threatening condition caused by a sudden infarction or hemorrhagic necrosis of the pituitary containing an adenoma. A wide variety of conditions can trigger apoplexy such as pituitary irradiation, general anesthesia, traumatic head injury, pituitary stimulatory tests and a wide variety of medications including bromocriptine. We report a case of a 16-year-old male patient with puberty arrest harboring a macroprolactinoma, who developed a sudden clinical picture of pituitary apoplexy during the 12th month of treatment with cabergoline.     

Age-related and gender-related occurrence of pituitary adenomas

OBJECTIVE To evaluate the various types of pituitary adenomas according to sex and age group. Few studies have attempted such an analysis, and most have focused on specific age groups, especially children. Recent data suggest that the frequency of different types of pituitary adenomas varies according to age and sex. DESIGN A retrospective review of the records of 2230 patients who underwent surgery for a pituitary adenoma at the University of California, San Francisco (UCSF), between January 1969 and June 1993. METHODS The distribution of tumours was analysed according to age at surgery, sex, and the clinical pheno-type of the tumour. Age groups were defined as the decades of life. RESULTS Prolactinomas were the most common tumours (39%), followed by endocrlne-inactive adenomas, growth-hormone-releasing adenomas, and adrenocorficotrophic hormone-releasing adenomas causing Cushing's disease; ACTH-releasing adenomas causing Nelson's syndrome and thyrotrophin (TSH)-releasing adenomas were rare. The female-to-male ratio differed considerably between the various adenoma types and between age groups. Prolactinomas, ACTH-releasing adenomas, and TSH-releasing adenomas occurred mostly in females; endocrine-inactive and GH-releasing adenomas occurred mostly in males. In older age groups, all adenoma types, except for endocrine-inactive adenomas, tended to assume a more balanced gender distribution. Among patients with prolactinomas, endocrine-inactive, ACTH-releasing, and to a lesser extent GH-releasing adenomas, the greatest discrepancy in gender distribution seemed to coincide with the peak Occurrence of each tumour type. The peak Occurrence was from the second to the fifth decade of life for prolactinomas and from the fourth to the eighth decade for endocrine-inactlve adenomas. GH-releasing, ACTH-releasing, and TSH-releaslng adenomas were more evenly distributed throughout the adult life span. CONCLUSIONS The frequency of pitultary adenomas varies greatly according to age and sex. The various adenoma types have thelr peak occurrence in distinctly dlfferent age groups and differ greatly in their female-to-male ratios. The female-to-male ratio for a given adenoma type varies greatly with age.     

Pituitary adenomas in childhood and adolescence. Clinical analysis of 10 cases

Pituitary adenomas in childhood and adolescence constitute 2-6% of all operated pituitary adenomas. We report the clinical features, treatment and follow-up of 10 pediatric patients affected by pituitary adenomas. All patients underwent clinical evaluation, endocrine tests, magnetic resonance imaging and visual field assessment. Follow-up ranged from 8 to 132 months (median 52.6). All patients were older than 10 years of age; 60% were males. In 50% the initial complaints were headache and/or visual impairment, all except one had clear evidence of endocrine dysfunction. Ninety percent were macroadenomas. According to hormone measurements and immunostaining 50% were prolactinomas, 20% were pure GH-secreting and 30% were non-functioning adenomas. Prolactinomas in two females were successfully treated with cabergoline. The other patients underwent surgery: three prolactinomas are still being treated with dopamine agonists and a GH-secreting adenoma is being treated with octreotide LAR and cabergoline. Two patients were also treated with conventional radiotherapy. Treatments were completely successful in 50% of patients: these have normal hormone secretion, full pubertal development, no significant tumor mass and normal visual field. Hypersecretion of prolactin persists in two cases; partial or complete hypopituitarism is present in four, relevant tumor remnant in another four and impairment of visual field is present in two cases. In conclusion, pediatric adenomas occur mostly in pubertal age, are prevalently macroadenomas and clinically functioning. Medical therapy should be preferred for secreting adenomas, but in some cases, notably prolactinomas in males, surgery and eventual radiotherapy may be needed.     

High prevalence of pituitary adenomas: a cross-sectional study in the province of Liege, Belgium

CONTEXT: Prevalence data are important for assessing the burden of disease on the health care system; data on pituitary adenoma prevalence are very scarce. OBJECTIVE: The objective of the study was to measure the prevalence of clinically relevant pituitary adenomas in a well-defined population. DESIGN: This was a cross-sectional, intensive, case-finding study performed in three regions of the province of Liège, Belgium, to measure pituitary adenoma prevalence as of September 30, 2005. SETTING: The study was conducted in specialist and general medical practitioner patient populations, referral hospitals, and investigational centers. METHODS: Three demographically and geographically distinct districts of the province of Liège were delineated precisely using postal codes. Medical practitioners in these districts were recruited, and patients with pituitary adenomas under their care were identified. Diagnoses were confirmed after retrieval of clinical, hormonal, radiological, and pathological data; full demographic and therapeutic follow-up data were collected in all cases. RESULTS: Sixty-eight patients with clinically relevant pituitary adenomas were identified in a population of 71,972 individuals; the mean (+/- sd) prevalence was 94 +/- 19.3 cases per 100,000 population (95% confidence interval, 72.2 to 115.8). The group was 67.6% female and had a mean age at diagnosis of 40.3 yr; 42.6% had macroadenomas and 55.9% underwent surgery. Prolactinomas comprised 66% of the group, with the rest having nonsecreting tumors (14.7%), somatotropinomas (13.2%), or Cushing's disease (5.9%); 20.6% had hypopituitarism. CONCLUSION: The prevalence of pituitary adenomas in the study population (one case in 1064 individuals) was more than 3.5-5 times that previously reported. This increased prevalence may have important implications when prioritizing funding for research and treatment of pituitary adenomas.     

Endoscopic endonasal transsphenoidal surgery: surgical results of 228 pituitary adenomas treated in a pituitary center

Pituitary tumors are challenging tumors in the sellar region. Surgical approaches to the pituitary have undergone numerous refinements over the last 100 years. The introduction of the endoscope have revolutionized pituitary surgery. The aim of this study is to report the results of a consecutive series of patients undergoing pituitary surgery using a pure endoscopic endonasal approach and to evaluate the efficacy and safety of this procedure. We reviewed the data of 228 consecutive patients who underwent endonasal transsphenoidal adenoma removal over an 10-year period. Pre- and post-operative hormonal status (at least 3 months after surgery) were analyzed and compared with clinical parameters presented by the patients. Tumor removal rate, endocrinological outcomes, and complications were retrospectively assessed in 228 patients with pituitary adenomas who underwent 251 procedures between December 1998 and December 2007. There were 93 nonfunctioning adenomas, 58 growth hormone-secreting, 41 prolactin-secreting, 28 adrenocorticotropin hormone secreting, 7 FSH-LH secreting and 1 thyroid-stimulating hormone-secreting adenomas. Gross total removal was achieved in 79.3% of the cases after a median follow-up of 61.5 months. The remission results for patients with nonfunctioning adenomas was 83% and for functioning adenomas were 76.3% (70.6% for GH hormone-secreting, 85.3% for prolactin hormone-secreting, 71.4% for ACTH hormone-secreting, 85.7% for FSH-LH hormone-secreting and 100% for TSH hormone-secreting), with no recurrence at the time of the last follow-up. Post-operative complications were present in 35 (13.9%) cases. The most frequent complications were temporary and permanent diabetes insipidus (six and two cases, respectively), syndrome of inappropriate antidiuretic hormone secretion (two cases) and CSF leaks (eight cases). There was no death related to the procedure in this series. The endoscopic endonasal approach for resection of pituitary adenomas, provides acceptable results representing a safe alternative procedure to the microscopic approach. This less invasive method, associated with a small number of complications, provides excellent tumor removal rates and represents an important tool for the achievement of good results in the pituitary surgery, mainly for the complete removal of large adenomas.     

经蝶入路显微外科治疗老年人垂体腺瘤

目的探讨老年人垂体腺瘤（ＳＰＡ）的手术疗效。方法总结经ＣＴ扫描或ＭＲＩ确诊的５６例ＳＰＡ经蝶显微外科治疗的临床资料，３５例经唇下－鼻中隔－蝶窦入路、２１例经鼻前庭－鼻中隔－蝶窦入路行肿瘤切除术。结果本组微腺瘤７例，大腺瘤３０例，巨大腺瘤１９例，术后无死亡。４８例获长期随访（平均３．５年），４１例恢复良好；７例术后平均３．４年肿瘤复发，其中２例再次手术，５例采用药物、放疗或放射外科治疗，于３个月时复查见患者恢复较为满意。结论采取经蝶显微外科治疗ＳＰＡ，是一种安全、有较的方法     

Clinical characterization of familial isolated pituitary adenomas

CONTEXT: Familial pituitary adenomas occur rarely in the absence of multiple endocrine neoplasia type 1 (MEN1) and Carney complex (CNC). OBJECTIVE: Our objective was to characterize the clinical and genealogical features of non-MEN1/CNC familial isolated pituitary adenomas (FIPA). DESIGN AND SETTING: We conducted a retrospective study of clinical and genealogical characteristics of FIPA cases and performed a comparison with a sporadic population at 22 university hospitals in Belgium, Italy, France, and The Netherlands. RESULTS: Sixty-four FIPA families including 138 affected individuals were identified [55 prolactinomas, 47 somatotropinomas, 28 nonsecreting adenomas (NS), and eight ACTH-secreting tumors]. Cases were MEN1/PRKAR1A-mutation negative. First-degree relationships predominated (75.6%) among affected individuals. A single tumor phenotype occurred in 30 families (homogeneous), and heterogeneous phenotypes occurred in 34 families. FIPA cases were younger at diagnosis than sporadic cases (P = 0.015); tumors were diagnosed earlier in the first vs. the second generation of multigenerational families. Macroadenomas were more frequent in heterogeneous vs. homogeneous FIPA families (P = 0.036). Prolactinomas from heterogeneous families were larger and had more frequent suprasellar extension (P = 0.004) than sporadic cases. Somatotropinomas occurred as isolated familial somatotropinoma cases and within heterogeneous FIPA families; isolated familial somatotropinoma cases represented 18% of FIPA cases and were younger at diagnosis than patients with sporadic somatotropinomas. Familial NS cases were younger at diagnosis (P = 0.03) and had more frequently invasive tumors (P = 0.024) than sporadic cases. CONCLUSIONS: Homogeneous and heterogeneous expression of prolactinomas, somatotropinomas, NS, and Cushing's disease can occur within families in the absence of MEN1/CNC. FIPA and sporadic cases have differing clinical characteristics. FIPA may represent a novel endocrine neoplasia classification that requires further genetic characterization.     

老年人垂体腺瘤49例术后临床分析

目的 探讨老年人垂体腺瘤的临床特点和治疗效果。方法 回顾分析49例手术及病理证实的老年人垂体腺瘤资料。结果 49例患者平均病程4．4年。视力视野障碍46例（93．8％）,头痛20例（40．8％）,内分泌障碍16例（32．7％）。肿瘤大于3cm25例（51．0％）,大部及部分切除11例（22．4％）,以经碾人路效果为好。术后常规放疗28例,肿瘤控制率93．0％,其中50％发生垂体功能低下。随访7－127个月（平均50．5个月）,视力改善27例（58．7％）,生活理43例（87＝8％）;40例术后3个月－5年复查CT或MRI提示肿瘤消失30例（61．2％）,残瘤静止5例（10．2％）,复发5例（10．2％）。结论 老年人垂体腺瘤以大腺瘤和无功能腺瘤为多;临床以视力视野改变为主;经碟人路显微手术是首选治疗方法;术后辅以放疗可控制或延缓肿瘤复发,但需注意视力损害及垂体功能低下的严重并发症。     

Acromegaly: a Latin American perspective

Pituitary adenomas in childhood and adolescence are relatively rare. In the present study we investigated intratumoral hemorrhage in pituitary adenomas and examined cases of intratumoral hemorrhage using adult patients for comparison. From 1975 to 2012, 38 consecutive patients operated for pituitary adenoma and one patient treated with medication alone, were enrolled in this study. Their ages were less than 18 years old at the initial diagnosis (mean age 15.3 ± 2.9 years). The comparison group consisted of 209 consecutive adult patients (>18 years old). The incidence and characteristics of intratumoral hemorrhage in pituitary adenomas were evaluated, based on magnetic resonance imaging (MRI) findings (28 cases) and on operative findings. The incidence of pituitary adenomas in childhood and adolescence was 38/1,073 (3.5 %) patients operated. Functioning pituitary adenomas (82.1 %) were common and non-functioning pituitary adenomas (17.9 %) were rare. Although no significant difference in tumor size was found and Knosp grade did not differ between young (≤18 years old) and adult (>18 years old) patients, indications of intratumoral hemorrhage on MRI was common in young patients (42.9 %). Based on both MRI and operative findings, intratumoral hemorrhage was significantly more likely to occur in young patients, compared with adult patients.     

Clinical features of nonpituitary sellar lesions in a large surgical series

Context Pituitary adenomas are the most common lesions in the sellar region, but other pathologies need to be considered in the differential diagnosis. Objective To assess the prevalence of unusual sellar masses in a large series of patients and identify clinicopathological factors that may aid the pre‐operative diagnosis. Design Retrospective case series. Patients We analysed the records of 1469 transsphenoidal procedures performed between 1998 and 2009. One hundred sixteen cases (7·9%) were not pituitary adenomas. Measurements Final pathological diagnosis. Results One hundred sixteen patients (45 men, 71 women; mean age (±SD): 45 ± 17 years) with nonadenomatous lesions were divided into four major aetiological groups: cystic lesions (CYS) (53%); benign neoplasms (BEN) (22%); malignancies (MAL) (16%) and inflammatory lesions (INF) (9%). Rathke’s cysts, the most common lesions, represented 42% of all cases. Twenty‐five per cent of malignant lesions were metastases, and some of the MAL (e.g., fibrosarcoma, lung metastasis) had a radiographical appearance suggestive of a pituitary adenoma. The most common presenting symptoms were visual field impairment (51%) and headache (34%). Pre‐operative pituitary dysfunction was present in 58% of cases, with hyperprolactinaemia (35%), hypogonadism (23%) and hypocortisolism (23%) found most frequently. Postoperative resolution of headache and visual symptoms occurred in 63% and 65% of patients, respectively. Hyperprolactinaemia resolved in 77% of cases. Conclusions A substantial minority of sellar masses are not pituitary adenomas. While they frequently present with the symptoms, hormone abnormalities and radiographical appearance typical of pituitary tumours, the possibility of a nonadenomatous lesion needs to be considered in the differential diagnosis.