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Serum IGF-1 May be less reliable when assessing the GH-IGF-1 axis in the elderly. 血清IGF-1在评估老年人GH-IGF-1轴时可能不太可靠。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-09-20 DOI: 10.1007/s11102-025-01575-4
Angelo Milioto, Gudmundur Johannsson, Daniela Esposito
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引用次数: 0
Silent growth hormone pituitary adenomas: a single-center study. 无声生长激素垂体腺瘤:一项单中心研究。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-09-11 DOI: 10.1007/s11102-025-01574-5
Tongxin Xiao, Xinxin Mao, Xueqing Zheng, Ran Li, Linjie Wang, Yong Yao, Kan Deng, Fengying Gong, Hui Pan, Lin Lu, Huijuan Zhu, Lian Duan

Purpose: To characterize the clinicopathological features and metabolic profiles of silent growth hormone pituitary adenomas (SGH-PAs).

Methods: The single-center retrospective study identified SGH-PAs from 1387 surgically treated non-functioning PAs at Peking Union Medical College Hospital (2019-2023). Controls included 407 consecutive cases with non-functioning gonadotropin pituitary adenomas (NFG-PAs) and 90 age and gender-matched acromegaly patients. Baseline and postoperative hormonal assessments, pituitary imaging features, pathological classification, metabolic comorbidity, and follow-up outcome were collected and analyzed.

Results: 45 cases of SGH-PA (3.2%) were identified from 1387 non-functioning PAs. The pathological subtypes included 18 (40%) acidophil stem cell adenomas, 11(24%) immature PIT1-lineage tumors, 5 sparsely granulated GH tumors, 5 mammosomatotroph tumors, 4 mature plurihormonal PIT1 lineage tumors, and 2 plurihormonal pituitary tumors. Compared to controls, SGH-PAs showed modestly elevated proliferation (median Ki-67 3% vs. 2% in both acromegaly [p = 0.002] and NFG-PAs [p = 0.011]) without outstanding increases in tumor invasiveness (Knosp 3-4: 20-30% across groups) at baseline or 2-year recurrence. Baseline metabolic comorbidities paralleled NFG-PA profiles for hypertension (22%) and diabetes (8.9%), with reduced dyslipidemia prevalence (17.8% vs. 33.4%, p = 0.049). No case of SGH-PAs transforming into acromegaly was confirmed during 77 person-years of follow-up.

Conclusions: SGH-PAs represent approximately 3% of nonfunctioning PAs undergoing surgery. Multiple anterior pituitary hormones are commonly co-expressed. These tumors may be more proliferative, but the baseline aggressiveness in radiology and short-term recurrence rates are similar.

目的:探讨垂体腺瘤(SGH-PAs)的临床病理特征和代谢特征。方法:采用单中心回顾性研究,从2019-2023年北京协和医院1387例手术治疗的无功能PAs中鉴定出SGH-PAs。对照组包括407例连续无功能的促性腺激素垂体腺瘤(NFG-PAs)和90例年龄和性别匹配的肢端肥大症患者。收集和分析基线和术后激素评估、垂体成像特征、病理分类、代谢合并症和随访结果。结果:1387例非功能性pa中鉴定出45例SGH-PA(3.2%)。病理亚型包括嗜酸干细胞腺瘤18例(40%),未成熟PIT1谱系肿瘤11例(24%),稀疏颗粒生长激素瘤5例,乳腺生长发育瘤5例,成熟多激素PIT1谱系肿瘤4例,多激素垂体瘤2例。与对照组相比,SGH-PAs在基线或2年复发时表现出适度的增殖升高(肢端肥大症的中位Ki-67 %对2% [p = 0.002]和NFG-PAs [p = 0.011]),肿瘤侵袭性没有明显增加(Knosp 3-4:各组间20-30%)。基线代谢合并症与高血压(22%)和糖尿病(8.9%)的NFG-PA谱相似,血脂异常患病率降低(17.8% vs. 33.4%, p = 0.049)。在77人年的随访中,未发现SGH-PAs转化为肢端肥大症的病例。结论:SGH-PAs约占手术中无功能PAs的3%。多种垂体前叶激素通常共表达。这些肿瘤可能更具增生性,但放射学的基线侵袭性和短期复发率相似。
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引用次数: 0
Should children born small for gestational age without catch-up growth be tested for growth hormone deficiency? 出生时胎龄小而没有补偿性生长的儿童是否应该进行生长激素缺乏症检测?
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-09-11 DOI: 10.1007/s11102-025-01571-8
Gianluca Tornese
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引用次数: 0
The clinicopathological PANOMEN-3 classification predicts pituitary adenoma prognosis: a real-world retrospective single center study of a surgically treated cohort. 临床病理PANOMEN-3分类预测垂体腺瘤预后:一项手术治疗队列的真实世界回顾性单中心研究。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-09-07 DOI: 10.1007/s11102-025-01562-9
Sabrina Chiloiro, Antonella Giampietro, Raffaele Migliore, Chiara Palumbo, Penelope Giambò, Flavia Costanza, Pier Paolo Mattogno, Rosalinda Calandrelli, Tommaso Tartaglione, Liverana Lauretti, Mario Rigante, Marco Gessi, Simona Gaudino, Laura De Marinis, Antonio Bianchi, Francesco Doglietto, Alfredo Pontecorvi

Introduction: Pituitary adenomas (PAs) are generally benign neoplasms, though in rare cases may exhibit aggressive behavior. In 2024, the PANOMEN-3 workshop released a new clinical-pathological classification. The objective of this study was to examine the potential of the PANOMEN-3 classification to predict prognosis of PAs and guide treatment in our single center cohort of patients with PAs.

Patients and methods: A longitudinal, retrospective, observational study was performed on patients with a PA diagnosis. The PANOMEN 3 classification was applied to each patient 6 months after surgery. Resultant grades were correlated with surgical outcome, disease recurrence or progression.

Results: 289 patients were included. According to the PANOMEN-3 classification, 9 patients (3.1%) were designated as grade 0, 101 patients as grade 1 (34.9%), 140 patients as grade 2 (48.4%) and 39 patients as a grade 3 (13.5%). At last follow-up assessment, 186 patients were found to be disease-free (64.4%), 93 patients (32.5%) exhibited a stable residual, 9 patients (3.1%) had recurrence and/or progression of their PA. The risk of recurrent/residual disease was increased in grade 1 (OR: OR:1.4 95%IC: 1.2-1.7), grade 2 (OR:1.5 95%IC: 1.2-1.9) and grade 3 (OR:5.7 95%IC: 2.7-12.5). Grades 1, 2 and 3 were associated with a shorter disease-free survival interval as compared to those with a grade 0 PANOMEN-3 score.

Conclusion: The PANOMEN-3 score is useful in clinical practice, aiding physicians to better plan patient follow-up, as well as to manage residual disease and treatment strategies post-surgery.

简介:垂体腺瘤(PAs)通常是良性肿瘤,尽管在极少数情况下可能表现出侵袭性行为。2024年,PANOMEN-3研讨会发布了一种新的临床病理分类。本研究的目的是研究PANOMEN-3分类在单中心队列PAs患者中预测PAs预后和指导治疗的潜力。患者和方法:对诊断为前列腺癌的患者进行纵向、回顾性、观察性研究。患者术后6个月采用PANOMEN 3分级。结果分级与手术结果、疾病复发或进展相关。结果:共纳入289例患者。根据panoen -3分级,0级9例(3.1%),1级101例(34.9%),2级140例(48.4%),3级39例(13.5%)。在最后的随访评估中,186例患者无病(64.4%),93例患者(32.5%)表现出稳定的残余,9例患者(3.1%)出现复发和/或进展。1级(OR: 1.4 95%IC: 1.2-1.7)、2级(OR:1.5 95%IC: 1.2-1.9)和3级(OR:5.7 95%IC: 2.7-12.5)复发/残留疾病的风险增加。与panoen -3评分0级的患者相比,1、2和3级患者的无病生存期较短。结论:PANOMEN-3评分有助于临床医生更好地规划患者的随访,以及术后残留疾病的管理和治疗策略。
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引用次数: 0
Outcomes of stereotactic radiosurgery in nelson's syndrome: a systematic review and Meta-Analysis. 立体定向放射手术治疗纳尔逊综合征的疗效:系统回顾和荟萃分析。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-08-30 DOI: 10.1007/s11102-025-01568-3
Bardia Hajikarimloo, Salem M Tos, Ibrahim Mohammadzadeh, Mohammad Amin Habibi

Background: Nelson's syndrome (NS) is an uncommon but serious complication after bilateral adrenalectomy (BA) in patients with Cushing's disease (CD). Stereotactic radiosurgery (SRS) has increasingly been used as a treatment option for NS patients; however, its effectiveness and safety remain uncertain. This meta-analysis aims to assess the role of SRS in NS.

Methods: A systematic review and meta-analysis were conducted following PRISMA guidelines. PubMed, Embase, Scopus, and Web of Science were searched through June 11, 2025, for studies reporting outcomes of SRS in NS. Pooled estimates were calculated for local control (LC), endocrine remission (ER), new pituitary dysfunction (NPD), salvage treatment (ST), and adverse radiation effects (ARE).

Results: Seven studies with 122 patients were included. Pooled LC was 95% (95% CI: 89-99%), ER was 21% (95% CI: 13-30%), NPD was 20% (95% CI: 11-31%), ST was 4% (95% CI: 0-9%), and ARE was 0% (95% CI: 0-11%). Meta-regression analysis revealed that larger lesion volume, longer interval from prior resection, and higher pre-SRS ACTH levels were significantly associated with an increased risk of ARE.

Conclusion: SRS provides high local control rates and a low safety risk for NS patients, although endocrine remission remains limited. These results support SRS as a viable choice in multidisciplinary management, although further prospective studies are needed.

背景:纳尔逊综合征(NS)是库欣病(CD)患者双侧肾上腺切除术(BA)后少见但严重的并发症。立体定向放射手术(SRS)越来越多地被用作NS患者的治疗选择;然而,其有效性和安全性仍不确定。本荟萃分析旨在评估SRS在NS中的作用。方法:根据PRISMA指南进行系统评价和荟萃分析。PubMed, Embase, Scopus和Web of Science检索到2025年6月11日,报告nsrs结果的研究。计算局部控制(LC)、内分泌缓解(ER)、新发垂体功能障碍(NPD)、挽救治疗(ST)和放射不良反应(ARE)的汇总估计。结果:纳入7项研究,122例患者。合并LC为95% (95% CI: 89-99%), ER为21% (95% CI: 13-30%), NPD为20% (95% CI: 11-31%), ST为4% (95% CI: 0-9%), ARE为0% (95% CI: 0-11%)。meta回归分析显示,较大的病变体积、较长的切除间隔和较高的srs前ACTH水平与ARE风险增加显著相关。结论:SRS为NS患者提供了高的局部控制率和低的安全风险,尽管内分泌缓解仍然有限。这些结果支持SRS作为多学科管理的可行选择,尽管需要进一步的前瞻性研究。
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引用次数: 0
The feedback loop in pituitary surgery. 垂体手术中的反馈回路。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-08-30 DOI: 10.1007/s11102-025-01569-2
Hani J Marcus, Andrea Giustina
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引用次数: 0
Urinary incontinence as a novel and underrecognized clinical manifestation of prolactinoma in women. 尿失禁作为一种新的和未被充分认识的临床表现在妇女催乳素瘤。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-08-30 DOI: 10.1007/s11102-025-01567-4
Emre Hepşen, Fatih Çanaklı, Halil Durantaş, Burçak Cavnar Helvacı, Kubilay Sarıkaya, Erman Çakal, Sema Hepşen

Objective: Prolactin exerts numerous pleiotropic effects on various tissues, including smooth muscle. Urinary incontinence, particularly the urge subtype, is primarily associated with detrusor overactivity-the involuntary contractions of the bladder's smooth muscle. The aim of this study is to investigate the potential impact of prolactinoma on urinary incontinence (UI) in women.

Methods: This cross-sectional study included 64 patients with prolactinoma and 80 controls. UI was assessed using the International Consultation on Incontinence Questionnaire-Short Form. Based on this instrument, incontinence was categorized into three subtypes: urge urinary incontinence (UUI), stress urinary incontinence (SUI), and mixed urinary incontinence (MUI).

Results: The overall rate of UI was significantly higher in prolactinoma patients, with 20 cases (31.3%), compared to 6 cases (7.5%) in the control group (p < 0.001). Compared to the control group, prolactinoma patients exhibited higher rates of UUI and MUI, while no difference was found in SUI rates (p = 0.012, p = 0.008, and p = 0.839; respectively). Incontinent patients had a higher mean age, a greater frequency of macroadenoma, and a longer disease duration compared to continent patients (p = 0.015, p = 0.038, and p < 0.001; respectively). The logistic regression analysis suggested that the disease duration was the only independent predictor of UI in the prolactinoma patients.

Conclusion: Prolactinoma, particularly in the later stages of the disease, may influence detrusor muscle activity in female patients and contribute to an increased prevalence of UI.

目的:催乳素对包括平滑肌在内的多种组织具有多种多效性作用。尿失禁,尤其是急迫性尿失禁,主要与逼尿肌过度活动有关——膀胱平滑肌的不自主收缩。本研究旨在探讨催乳素瘤对女性尿失禁(UI)的潜在影响。方法:本横断面研究纳入64例泌乳素瘤患者和80例对照组。使用国际失禁咨询问卷-短表格评估尿失禁。基于该仪器,将尿失禁分为三种亚型:急迫性尿失禁(UUI)、压力性尿失禁(SUI)和混合性尿失禁(MUI)。结果:泌乳素瘤患者尿失禁的总体发生率明显高于对照组,为20例(31.3%),而对照组为6例(7.5%)。(p)结论:泌乳素瘤,特别是在疾病的晚期,可能影响女性患者的逼尿肌活动,导致尿失禁的患病率增加。
{"title":"Urinary incontinence as a novel and underrecognized clinical manifestation of prolactinoma in women.","authors":"Emre Hepşen, Fatih Çanaklı, Halil Durantaş, Burçak Cavnar Helvacı, Kubilay Sarıkaya, Erman Çakal, Sema Hepşen","doi":"10.1007/s11102-025-01567-4","DOIUrl":"10.1007/s11102-025-01567-4","url":null,"abstract":"<p><strong>Objective: </strong>Prolactin exerts numerous pleiotropic effects on various tissues, including smooth muscle. Urinary incontinence, particularly the urge subtype, is primarily associated with detrusor overactivity-the involuntary contractions of the bladder's smooth muscle. The aim of this study is to investigate the potential impact of prolactinoma on urinary incontinence (UI) in women.</p><p><strong>Methods: </strong>This cross-sectional study included 64 patients with prolactinoma and 80 controls. UI was assessed using the International Consultation on Incontinence Questionnaire-Short Form. Based on this instrument, incontinence was categorized into three subtypes: urge urinary incontinence (UUI), stress urinary incontinence (SUI), and mixed urinary incontinence (MUI).</p><p><strong>Results: </strong>The overall rate of UI was significantly higher in prolactinoma patients, with 20 cases (31.3%), compared to 6 cases (7.5%) in the control group (p < 0.001). Compared to the control group, prolactinoma patients exhibited higher rates of UUI and MUI, while no difference was found in SUI rates (p = 0.012, p = 0.008, and p = 0.839; respectively). Incontinent patients had a higher mean age, a greater frequency of macroadenoma, and a longer disease duration compared to continent patients (p = 0.015, p = 0.038, and p < 0.001; respectively). The logistic regression analysis suggested that the disease duration was the only independent predictor of UI in the prolactinoma patients.</p><p><strong>Conclusion: </strong>Prolactinoma, particularly in the later stages of the disease, may influence detrusor muscle activity in female patients and contribute to an increased prevalence of UI.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"28 5","pages":"96"},"PeriodicalIF":3.4,"publicationDate":"2025-08-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144966023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Semaglutide treatment in hypothalamic obesity: Two-Year outcomes on body composition, appetite, and quality of life. 下丘脑肥胖的西马鲁肽治疗:身体组成、食欲和生活质量的两年结局。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-08-19 DOI: 10.1007/s11102-025-01564-7
Erlend Gjersdal, Frederik Østergaard Klit, Kåre Schmidt Ettrup, Peter Vestergaard, Eigil Husted Nielsen, Kristian Nilsson Vistisen, Hermann L Müller, Dorte Melgaard, Jakob Dal

Purpose: Hypothalamic obesity is a severe complication of craniopharyngioma, marked by hyperphagia and rapid weight gain. Glucagon-like peptide-1 receptor agonists such as semaglutide have shown promising effects on weight reduction, but long-term data on weight outcomes, metabolic parameters and quality-of-life remain limited.

Methods: Four female patients with hypothalamic obesity following craniopharyngioma treatment received semaglutide for 24 months. Assessments included DXA scans, metabolic biomarkers, and The Three-Factor Eating Questionnaire at baseline, 6, 12, and 24 months. Interviews at 24 months explored hunger, side effects and quality of life.

Results: After 24 months, median weight loss was 16% (95% CI: 8 to 34%, p = 0.004), with maximal loss of 17% at 6 months. Emotional and uncontrolled eating scores (range: 0-100) decreased by 44 (95% CI: - 69 to - 19, p = 0.011) and 27 units (95% CI: - 63 to 9, p = 0.097), respectively. Interviews revealed reduced hunger, improved self-confidence, less isolation, and higher productivity. Treatment was well tolerated; side effects were mainly mild GI symptoms. Fat and lean mass decreased by 10% (95% CI: 2 to 44%, p = 0.016) and 19% (95% CI: 14 to 26%, p < 0.001), respectively, with stable bone mineral content. Hemoglobin A1c and LDL cholesterol declined by 6.4 mmol/mol (95% CI: 2.3 to 9.9, p = 0.016) and 0.5 mmol/L (95% CI: 0.3 to 0.7, p = 0.012).

Conclusion: Semaglutide is a safe treatment that led to long-term sustained improvements in eating behavior, weight control, and improved metabolic health. Patients reported an improved quality of life, which persisted after body weight stabilization.

目的:下丘脑肥胖是颅咽管瘤的严重并发症,其特点是嗜食和体重迅速增加。胰高血糖素样肽-1受体激动剂(如semaglutide)已显示出有希望的减肥效果,但关于体重结局、代谢参数和生活质量的长期数据仍然有限。方法:4例颅咽管瘤治疗后下丘脑肥胖的女性患者给予西马鲁肽治疗24个月。评估包括基线、6个月、12个月和24个月的DXA扫描、代谢生物标志物和三因素饮食问卷。24个月时的访谈探讨了饥饿、副作用和生活质量。结果:24个月后,中位体重减轻16% (95% CI: 8 - 34%, p = 0.004), 6个月时最大体重减轻17%。情绪化和不受控制的饮食得分(范围:0-100)分别下降了44 (95% CI: - 69至- 19,p = 0.011)和27 (95% CI: - 63至9,p = 0.097)。采访显示,饥饿感减少,自信心增强,孤独感减少,工作效率提高。治疗耐受性良好;副作用主要是轻微的胃肠道症状。脂肪和瘦肉质量分别下降10% (95% CI: 2 - 44%, p = 0.016)和19% (95% CI: 14 - 26%, p)。结论:Semaglutide是一种安全的治疗方法,可导致饮食行为、体重控制和代谢健康的长期持续改善。患者报告生活质量得到改善,这种改善在体重稳定后持续存在。
{"title":"Semaglutide treatment in hypothalamic obesity: Two-Year outcomes on body composition, appetite, and quality of life.","authors":"Erlend Gjersdal, Frederik Østergaard Klit, Kåre Schmidt Ettrup, Peter Vestergaard, Eigil Husted Nielsen, Kristian Nilsson Vistisen, Hermann L Müller, Dorte Melgaard, Jakob Dal","doi":"10.1007/s11102-025-01564-7","DOIUrl":"10.1007/s11102-025-01564-7","url":null,"abstract":"<p><strong>Purpose: </strong>Hypothalamic obesity is a severe complication of craniopharyngioma, marked by hyperphagia and rapid weight gain. Glucagon-like peptide-1 receptor agonists such as semaglutide have shown promising effects on weight reduction, but long-term data on weight outcomes, metabolic parameters and quality-of-life remain limited.</p><p><strong>Methods: </strong>Four female patients with hypothalamic obesity following craniopharyngioma treatment received semaglutide for 24 months. Assessments included DXA scans, metabolic biomarkers, and The Three-Factor Eating Questionnaire at baseline, 6, 12, and 24 months. Interviews at 24 months explored hunger, side effects and quality of life.</p><p><strong>Results: </strong>After 24 months, median weight loss was 16% (95% CI: 8 to 34%, p = 0.004), with maximal loss of 17% at 6 months. Emotional and uncontrolled eating scores (range: 0-100) decreased by 44 (95% CI: - 69 to - 19, p = 0.011) and 27 units (95% CI: - 63 to 9, p = 0.097), respectively. Interviews revealed reduced hunger, improved self-confidence, less isolation, and higher productivity. Treatment was well tolerated; side effects were mainly mild GI symptoms. Fat and lean mass decreased by 10% (95% CI: 2 to 44%, p = 0.016) and 19% (95% CI: 14 to 26%, p < 0.001), respectively, with stable bone mineral content. Hemoglobin A1c and LDL cholesterol declined by 6.4 mmol/mol (95% CI: 2.3 to 9.9, p = 0.016) and 0.5 mmol/L (95% CI: 0.3 to 0.7, p = 0.012).</p><p><strong>Conclusion: </strong>Semaglutide is a safe treatment that led to long-term sustained improvements in eating behavior, weight control, and improved metabolic health. Patients reported an improved quality of life, which persisted after body weight stabilization.</p>","PeriodicalId":20202,"journal":{"name":"Pituitary","volume":"28 5","pages":"93"},"PeriodicalIF":3.4,"publicationDate":"2025-08-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12364737/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144883513","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Evaluating pituitary tumor management: aligning with pituitary tumor centers of excellence criteria. 评价垂体肿瘤的管理:与垂体肿瘤卓越中心的标准一致。
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-08-14 DOI: 10.1007/s11102-025-01563-8
Jin-Han Yang, Wei-Hsin Wang, Harn-Shen Chen

Background: The Pituitary Society established criteria to develop Pituitary Tumor Centers of Excellence (PTCOE) for optimization of patient care. These criteria were later transformed into quantitative standards based on real-life data from accredited pituitary centers worldwide. The aim of this study was to evaluate the pituitary tumor care capacity at our institute and compare it with the PTCOE criteria.

Method: We retrospectively reviewed the data of patients who underwent sellar, suprasellar, or parasellar surgery during 2021-2023 at Taipei Veterans General Hospital. Adults older than 18 years who were diagnosed with pituitary tumors were included. We collected data regarding baseline patient and tumor characteristics, the surgical method, complications, and activity volumes across involved departments. The relevant data were compared with those of the PTCOE standards.

Results: In total, 182 patients with pituitary tumors underwent surgery via the endoscopic endonasal approach during 2021-2023. Their median age was 51 (range, 20-91) years. Among them, 90.7% had macroadenomas. Functional remission rates were 55.6% for acromegaly, 69.2% for prolactinoma, and 93.3% for Cushing's disease. Our institute met the acceptable PTCOE criteria for the number of pituitary interventions, postoperative readmissions for complications, dynamic endocrine tests, neuroradiologists, and neuro-oncologists. Further, we met the preferred PTCOE criteria for mortality rate and the numbers of dedicated surgeons, endocrinologists, trained nurses, neuropathologists, and neuro-ophthalmologists.

Conclusion: Most indicators in our study met the acceptable standards for a PTCOE. During the study period, the multidisciplinary team at our institute collaborated closely to provide comprehensive care for patients with pituitary adenomas.

背景:垂体学会建立标准,发展垂体肿瘤卓越中心(PTCOE),以优化患者护理。这些标准后来被转化为定量标准,基于来自世界各地经认可的垂体中心的真实数据。本研究的目的是评估我院垂体肿瘤护理能力,并与PTCOE标准进行比较。方法:我们回顾性分析台北荣民总医院2021-2023年间接受鞍、鞍上或鞍旁手术的患者资料。年龄在18岁以上被诊断为垂体瘤的成年人也包括在内。我们收集了有关基线患者和肿瘤特征、手术方法、并发症和相关科室活动量的数据。将相关数据与PTCOE标准进行比较。结果:在2021-2023年期间,共有182例垂体肿瘤患者经鼻内镜入路手术。他们的中位年龄为51岁(20-91岁)。其中90.7%为大腺瘤。肢端肥大症的功能缓解率为55.6%,泌乳素瘤为69.2%,库欣病为93.3%。在垂体干预、术后并发症再入院、动态内分泌测试、神经放射科医生和神经肿瘤科医生的数量方面,我们研究所达到了可接受的PTCOE标准。此外,我们在死亡率和专业外科医生、内分泌学家、训练有素的护士、神经病理学家和神经眼科医生的数量方面达到了首选的PTCOE标准。结论:本研究大部分指标均符合PTCOE可接受标准。在研究期间,我院多学科团队密切合作,为垂体腺瘤患者提供全面的护理。
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引用次数: 0
Genetic evaluation of pediatric pituitary adenomas and USP8-related genotype-phenotype correlations in Cushing's disease. 库欣病儿童垂体腺瘤的遗传评价及usp8相关基因型-表型相关性
IF 3.4 2区 医学 Q2 ENDOCRINOLOGY & METABOLISM Pub Date : 2025-08-14 DOI: 10.1007/s11102-025-01557-6
Rida Zainab, Sukhvir Kaur, Justin Lack, Morgan Similuk, Mayank Tandon, Rajarshi Ghosh, Bryce A Seifert, Mari Tokita, Chelsi Flippo, Jia Yan, Magdalena Walkiewicz, Prashant Chittiboina, Christina Tatsi

Purpose: Pituitary adenomas (PAs) constitute a rare pediatric diagnosis and their pathogenetic mechanisms are not clearly understood. The aim of this study was to evaluate the prevalence of genetic defects in pediatric PAs through germline and tumor testing, and to describe genotype-phenotype correlations.

Methods: Fifty-four pediatric patients with PAs and available germline and/or tumor samples were studied. Germline and/or tumor sequencing were reviewed for variants in genes previously associated with pituitary tumorigenesis.

Results: Germline genetic testing revealed a pathogenic variant in AIP gene in 2 patients with growth hormone excess (GHE) and a likely pathogenic variant in CDKN2A in a patient with Cushing's disease (CD). Somatic gene sequencing identified pathogenic variants in GNAS in 4/7 patients (57.1%) with GHE. 6/38 patients (15.8%) with CD had pathogenic variants in USP8 gene, and in one tumor pathogenic variants in PRKAR1A, TP53 and MEN1 genes were identified. Overall, pathogenic/likely pathogenic germline or somatic variants were identified in 14/54 patients (25.9%). When evaluating the genotype-phenotype correlations in patients with CD, patients with somatic USP8 pathogenic variants had larger tumors (median size: 9.5 mm [6.5, 13.3] vs. 6 mm [4.0, 7.0], p = 0.048), trend towards higher incidence of cavernous sinus invasion (50% vs. 12.5%, p = 0.06), and higher risk of non-remission after surgery (33.3% vs. 0%, p = 0.021) compared to patients without USP8 variants.

Conclusions: Somatic USP8 pathogenic variants correlate with worse tumor behavior and patient outcomes in pediatric-onset CD. Unlike GH-secreting PAs, the genetic basis of the majority of pediatric corticotroph PAs remains unclear. Further studies are needed to explore the genetic drivers of pediatric CD.

Clinicaltrials:

Gov id: NCT00001595, NCT03206099.

目的:垂体腺瘤(PAs)是一种罕见的儿科诊断,其发病机制尚不清楚。本研究的目的是通过种系和肿瘤检测来评估儿科PAs遗传缺陷的患病率,并描述基因型-表型相关性。方法:对54例儿科PAs患者和可用的种系和/或肿瘤样本进行研究。我们回顾了生殖系和/或肿瘤测序,以寻找先前与垂体肿瘤发生相关的基因变异。结果:生殖系基因检测在2例生长激素过量(GHE)患者中发现了AIP基因的致病性变异,在1例库欣病(CD)患者中发现了CDKN2A可能的致病性变异。体细胞基因测序在4/7 (57.1%)GHE患者中鉴定出GNAS致病性变异。6/38例CD患者(15.8%)存在USP8基因的致病变异,其中PRKAR1A、TP53和MEN1基因的肿瘤致病变异1例。总体而言,54例患者中有14例(25.9%)发现致病性/可能致病性种系或体细胞变异。在评估CD患者的基因型-表型相关性时,与没有USP8变异的患者相比,有体细胞USP8致病变异的患者肿瘤更大(中位尺寸:9.5 mm[6.5, 13.3]对6 mm [4.0, 7.0], p = 0.048),海绵窦侵袭发生率更高(50%对12.5%,p = 0.06),术后不缓解的风险更高(33.3%对0%,p = 0.021)。结论:在儿科发病的CD中,躯体USP8致病变异与更糟糕的肿瘤行为和患者预后相关。与分泌gh的PAs不同,大多数儿童促皮质性PAs的遗传基础尚不清楚。需要进一步的研究来探索儿科cd的遗传驱动因素。临床试验:NCT00001595, NCT03206099。
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引用次数: 0
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