Pituitary Neoplasms

垂体肿瘤
  • 文章类型: Journal Article
    垂体腺瘤在19岁以下的儿童和年轻人中很少见(以下称为CYP),但与成年人相比,它们在该年龄段的诊断和管理方面存在一些不同的挑战。这些罕见的肿瘤可以破坏成熟,视觉,智力和发展过程,在CYP,他们倾向于有更多的隐秘性表现,攻击性行为,比成年人更有可能有遗传基础。通过标准化的AGREEII方法,文献综述和德尔菲共识,一个多学科专家组制定了74项务实的管理建议,旨在优化CYP的治疗,这是首个全面共识指南,涵盖CYP伴垂体腺瘤的治疗.该共识指南的第2部分详细介绍了针对儿科泌乳素瘤患者的57条建议,库欣病,生长激素过量导致巨人症和肢端肥大症,临床无功能腺瘤,还有罕见的TSHOMA.与成人垂体腺瘤患者相比,我们强调,在CYP组中,有更大比例的功能性肿瘤,包括大型泌乳素瘤,潜在遗传疾病的可能性更大,10岁以下男孩的促肾上腺皮质激素瘤多于女孩,并且难以诊断生长激素过量。与照顾成年患者的垂体专家合作,作为委托和集中的多学科团队的一部分,是优化管理的关键,过渡和终身护理,并促进收集与健康相关的生存质量,手术和放射治疗,目前大部分缺失。
    Pituitary adenomas are rare in children and young people under the age of 19 (hereafter referred to as CYP) but they pose some different diagnostic and management challenges in this age group than in adults. These rare neoplasms can disrupt maturational, visual, intellectual and developmental processes and, in CYP, they tend to have more occult presentation, aggressive behaviour and are more likely to have a genetic basis than in adults. Through standardized AGREE II methodology, literature review and Delphi consensus, a multidisciplinary expert group developed 74 pragmatic management recommendations aimed at optimizing care for CYP in the first-ever comprehensive consensus guideline to cover the care of CYP with pituitary adenoma. Part 2 of this consensus guideline details 57 recommendations for paediatric patients with prolactinomas, Cushing disease, growth hormone excess causing gigantism and acromegaly, clinically non-functioning adenomas, and the rare TSHomas. Compared with adult patients with pituitary adenomas, we highlight that, in the CYP group, there is a greater proportion of functioning tumours, including macroprolactinomas, greater likelihood of underlying genetic disease, more corticotrophinomas in boys aged under 10 years than in girls and difficulty of peri-pubertal diagnosis of growth hormone excess. Collaboration with pituitary specialists caring for adult patients, as part of commissioned and centralized multidisciplinary teams, is key for optimizing management, transition and lifelong care and facilitates the collection of health-related quality of survival outcomes of novel medical, surgical and radiotherapeutic treatments, which are currently largely missing.
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  • 文章类型: Journal Article
    垂体前叶部分肿瘤仅占所有儿童(年龄<15岁)颅内肿瘤的1%,然而,他们可以提供高发病率和很少的证据和指导他们的管理。在2014年至2022年之间,一个多学科专家组系统地制定了第一个针对19岁以下儿童和年轻人的综合临床实践共识指南(以下简称CYP),提出了疑似垂体腺瘤,以告知专科护理并改善健康结果。通过强大的文献检索和与国际德尔福共识专家小组的德尔福共识练习,现有的科学证据和专家意见被合并为74条建议。本共识指南的第1部分包括17项与临床护理相关的务实管理建议,神经影像学,视觉评估,组织病理学,遗传学,垂体手术和放射治疗。虽然在许多方面,CYP的护理与成年人相似,存在关键差异,特别是在病因和表现方面。疑似垂体腺瘤的CYP需要仔细的临床检查,适当的荷尔蒙检查,专门的垂体成像和视觉评估。应考虑综合征和遗传评估的潜力。地方和国家层面的多学科讨论可能是管理的关键。手术应在专科中心进行。收集关于新的医疗方式的结果数据,手术干预和放射治疗对于未来的最佳治疗至关重要。
    Tumours of the anterior part of the pituitary gland represent just 1% of all childhood (aged <15 years) intracranial neoplasms, yet they can confer high morbidity and little evidence and guidance is in place for their management. Between 2014 and 2022, a multidisciplinary expert group systematically developed the first comprehensive clinical practice consensus guideline for children and young people under the age 19 years (hereafter referred to as CYP) presenting with a suspected pituitary adenoma to inform specialist care and improve health outcomes. Through robust literature searches and a Delphi consensus exercise with an international Delphi consensus panel of experts, the available scientific evidence and expert opinions were consolidated into 74 recommendations. Part 1 of this consensus guideline includes 17 pragmatic management recommendations related to clinical care, neuroimaging, visual assessment, histopathology, genetics, pituitary surgery and radiotherapy. While in many aspects the care for CYP is similar to that of adults, key differences exist, particularly in aetiology and presentation. CYP with suspected pituitary adenomas require careful clinical examination, appropriate hormonal work-up, dedicated pituitary imaging and visual assessment. Consideration should be given to the potential for syndromic disease and genetic assessment. Multidisciplinary discussion at both the local and national levels can be key for management. Surgery should be performed in specialist centres. The collection of outcome data on novel modalities of medical treatment, surgical intervention and radiotherapy is essential for optimal future treatment.
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  • 文章类型: English Abstract
    Craniopharyngioma is a sellar tumor with benign pathology and malignant clinical manifestations. Partial resection plus radiotherapy, internal radiation therapy and chemotherapy are the conventional treatment strategies forcraniopharyngioma in most hospitals, which cause many patients to lose the best time to cure and make reoperation more difficult after recurrence. Therefore, the Pediatric Group of Chinese Society of Neurosurgery, Chinese Medical Association organized domestic experts from Pediatrics, Pediatric Neurosurgery, Genetics and Endocrinology to reach a consensus on the following topics: pathological and molecular genetic classification of craniopharyngioma, basic principles of curing tumors, surgical classification of tumors, surgical approach, perioperative and long-term treatment plan. This is expected to improve the quality of life and prognosis of patients, and further standardize the diagnosis and treatment of craniopharyngioma.
    颅咽管瘤是良性病理、恶性临床表现的鞍区肿瘤。多数单位以部分切除+放疗、内照射治疗、化疗等作为颅咽管瘤治疗的常规策略,导致许多患者失去最佳治愈时机,复发后再次手术也更为困难。为此,中华医学会神经外科学分会小儿学组组织国内神经外科、儿科、小儿神经外科、遗传与内分泌科专家围绕以下主题达成共识:颅咽管瘤的病理学和分子遗传学分型、治愈肿瘤的基本原则、肿瘤的外科学分型、手术入路、围手术期和远期治疗方案,以期改善患者的生存质量及预后,进一步规范颅咽管瘤的诊治。.
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  • 文章类型: Journal Article
    垂体肿瘤(PT)占颅内肿瘤的15%,影响人口的10.7%-14.4%,尽管临床相关PT的发生率为5.1例/100,000居民。手术治疗适用于激素分泌过多的PT(产生催乳素的PT除外)和具有局部压迫或整体神经系统症状的PT。多学科护理,对PT患者至关重要,最好在卓越中心交付,并基于明确的护理方案。为了方便和规范这类肿瘤的临床操作,本文件收集了西班牙内分泌与营养学会(SEEN)和西班牙神经外科学会(SENEC)的神经内分泌学知识领域对PT患者及其术前管理的定位,手术和术后随访。
    Pituitary tumors (PT) account for 15% of intracranial tumors affect 10.7%-14.4% of the population although the incidence of clinically relevant PT is 5.1 cases/100,000 inhabitants. Surgical treatment is indicated in PTs with hormone hypersecretion (except for prolactin-producing PTs) and those with local compressive or global neurological symptoms. Multidisciplinary care, is essential for patients with PTs, preferably delivered in a center of excellence and based on a well-defined care protocol. In order to facilitate and standardize the clinical procedures for this type of tumor, this document gathers the positioning of the Neuroendocrinology Knowledge Area of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Neurosurgery (SENEC) on the management of patients with PTs and their preoperative, surgical and postoperative follow-up.
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  • 文章类型: Journal Article
    垂体肿瘤(PT)占颅内肿瘤的15%,影响人口的10.7%至14.4%,尽管临床相关PT的发生率为5.1例/100,000居民。手术治疗适用于激素分泌过多的PT(产生催乳素的PT除外)和具有局部压迫或整体神经系统症状的PT。多学科护理,对PT患者至关重要,最好在卓越中心交付,并基于明确的护理方案。为了方便和规范这类肿瘤的临床操作,本文件收集了西班牙内分泌与营养学会(SEEN)和西班牙神经外科学会(SENEC)的神经内分泌学知识领域对PT患者及其术前管理的定位,手术和术后随访。
    Pituitary tumors (PT) account for 15% of intracranial tumors affect 10.7-14.4% of the population although the incidence of clinically relevant PT is 5.1 cases/100,000 inhabitants. Surgical treatment is indicated in PTs with hormone hypersecretion (except for prolactin-producing PTs) and those with local compressive or global neurological symptoms. Multidisciplinary care, is essential for patients with PTs, preferably delivered in a center of excellence and based on a well-defined care protocol. In order to facilitate and standardize the clinical procedures for this type of tumor, this document gathers the positioning of the Neuroendocrinology Knowledge Area of the Spanish Society of Endocrinology and Nutrition (SEEN) and the Spanish Society of Neurosurgery (SENEC) on the management of patients with PTs and their preoperative, surgical and postoperative follow-up.
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  • 文章类型: Journal Article
    这份来自国际社会的共识声明,由垂体学会主办的多学科研讨会提供了基于证据的分级共识建议和关键总结点,用于泌乳素瘤的诊断和管理的临床实践.流行病学和发病机制,垂体激素分泌紊乱的临床表现,高泌乳素血症的评估和生化评估,解决了影像学策略和疾病相关并发症的最佳使用。深入讨论提供有关治疗催乳素瘤的最新证据,包括功效,多巴胺激动剂治疗停药的不良反应和选择,以及手术的适应症,术前药物治疗和放射治疗。讨论了特殊情况下泌乳素瘤的管理,包括囊性病变,混合分泌生长激素和分泌催乳素的腺瘤和巨大和侵袭性泌乳素腺瘤。此外,概述了怀孕和生育的考虑因素,以及儿童和青少年泌乳素瘤的管理,患有潜在精神疾病的患者,绝经后妇女,变性人和慢性肾脏病患者。研讨会的结论是,尽管治疗耐药性很少见,需要额外的治疗方案来应对这些患者治疗的临床挑战,并且需要促进国际注册,以实现风险分层和治疗策略的优化.
    This Consensus Statement from an international, multidisciplinary workshop sponsored by the Pituitary Society offers evidence-based graded consensus recommendations and key summary points for clinical practice on the diagnosis and management of prolactinomas. Epidemiology and pathogenesis, clinical presentation of disordered pituitary hormone secretion, assessment of hyperprolactinaemia and biochemical evaluation, optimal use of imaging strategies and disease-related complications are addressed. In-depth discussions present the latest evidence on treatment of prolactinoma, including efficacy, adverse effects and options for withdrawal of dopamine agonist therapy, as well as indications for surgery, preoperative medical therapy and radiation therapy. Management of prolactinoma in special situations is discussed, including cystic lesions, mixed growth hormone-secreting and prolactin-secreting adenomas and giant and aggressive prolactinomas. Furthermore, considerations for pregnancy and fertility are outlined, as well as management of prolactinomas in children and adolescents, patients with an underlying psychiatric disorder, postmenopausal women, transgender individuals and patients with chronic kidney disease. The workshop concluded that, although treatment resistance is rare, there is a need for additional therapeutic options to address clinical challenges in treating these patients and a need to facilitate international registries to enable risk stratification and optimization of therapeutic strategies.
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  • 文章类型: Journal Article
    虽然罕见,颅咽管瘤占儿童期下丘脑-垂体区肿瘤的80%。尽管是良性的,这些肿瘤非常接近视觉通路,下丘脑,和垂体腺意味着在高总生存率的背景下,肿瘤的治疗和肿瘤本身都可以导致明显的长期神经内分泌发病率。迄今为止,这些肿瘤的最佳管理策略仍然不确定,各中心之间的实践各不相同。鉴于这些差异,作为国家努力为英国罕见儿科内分泌肿瘤的管理创建基于证据和基于共识的指导的一部分,我们的目标是制定指导方针,这些都在这篇评论中介绍。这些指南是在英国儿童癌症和白血病小组和英国儿科内分泌和糖尿病学会的主持下制定的,在皇家儿科和儿童健康学院的监督和认可下,使用《研究与评估指南II》方法来标准化颅咽管瘤儿童和年轻人的护理。
    Although rare, craniopharyngiomas constitute up to 80% of tumours in the hypothalamic-pituitary region in childhood. Despite being benign, the close proximity of these tumours to the visual pathways, hypothalamus, and pituitary gland means that both treatment of the tumour and the tumour itself can cause pronounced long-term neuroendocrine morbidity against a background of high overall survival. To date, the optimal management strategy for these tumours remains undefined, with practice varying between centres. In light of these discrepancies, as part of a national endeavour to create evidence-based and consensus-based guidance for the management of rare paediatric endocrine tumours in the UK, we aimed to develop guidelines, which are presented in this Review. These guidelines were developed under the auspices of the UK Children\'s Cancer and Leukaemia Group and the British Society for Paediatric Endocrinology and Diabetes, with the oversight and endorsement of the Royal College of Paediatrics and Child Health using Appraisal of Guidelines for Research & Evaluation II methodology to standardise care for children and young people with craniopharyngiomas.
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  • 文章类型: Journal Article
    主要目的是根据第11号肢端肥大症共识声明(2018)分析短期和两年随访中的鼻内镜手术结果。的确,分析预后因素及并发症。
    分析了2013年至2020年期间接受鼻内镜手术并肢端肥大症的40例患者。如果在六个月和两年的随访中正常(ULN)IGF-1的上限小于1.0,则认为患者处于缓解状态。此外,我们评估了Knosp等级,肿瘤体积学,ULN,MRI中的T2信号,再操作,和并发症。
    入院平均年龄为46.7岁。32例患者在手术6个月后缓解(80%),在两年的随访中下降到76.32%。所有微腺瘤均缓解(n=6)。关于并发症,3例患者出现永久性全垂体功能减退症(7.5%);本系列中没有发生术后脑脊液(CSF)渗漏.在多因素Logistic回归分析中,T2MRI上的高信号和较高的肿瘤体积是不发射的单一预测因素(p<0.05)。术前激素水平(GH和IGF-1)不是缓解的预后因素。出现超信号的再次手术患者已经具有很高的临床手术失败预测因子。
    鼻内镜手术可促进肢端肥大症的短期和两年高缓解率;在非缓解患者中,肿瘤体积和T2高信号是有统计学意义的预后因素——与文献相比,并发症发生率相似。在侵袭性GH分泌肿瘤中,我们应该为这些患者提供多学科的方法来提高肢端肥大症患者的缓解率。
    UNASSIGNED: The primary aim is to analyze the endoscopic endonasal surgical results in short-term and two-year follow-ups according to the 11th Acromegaly Consensus statement (2018). Indeed, prognostic factors and complications were analyzed.
    UNASSIGNED: 40 patients who underwent endoscopic endonasal surgery by acromegaly between 2013 to 2020 was analyzed. Patients were considered in remission if an upper limit of normal (ULN) IGF-1 was less than 1.0 at the six-month and two-year follow-ups. Moreover, we assessed the Knosp grade, tumor volumetry, ULN, T2 signal in MRI, reoperation, and complications.
    UNASSIGNED: The mean age of admission was 46.7 years. Thirty-two patients were in remission after six months of surgery (80%), decreasing to 76.32% at the two-year follow-up. All microadenomas presented remission (n = 6). Regarding the complications, three patients had permanent panhypopituitarism (7.5%); postoperative cerebrospinal fluid (CSF) leaks did not occur in this series. The hyperintense signal on the T2 MRI and a higher tumor volumetry were the single predictor\'s factors of non-emission in a multivariate regression logistic analysis (p < 0.05). Preoperative hormone levels (GH and IGF-1) were not a prognostic factor for remission. The re-operated patients who presented hypersignal already had a high predictor of clinical-operative failure.
    UNASSIGNED: The endoscopic endonasal surgery promotes high short-term and two-year remission rates in acromegaly; the tumor\'s volumetry and the T2 hypersignal were statistically significant prognostic factors in non-remission - the complications presented at similar rates in comparison to the literature. In invasive GH-secreting tumors, we should offer these patients a multi-disciplinary approach to improve acromegalic patients\' remission rates.
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  • 文章类型: Journal Article
    背景:本指南(GL)旨在为成人泌乳素(PRL)分泌型垂体腺瘤的治疗提供参考。然而,怀孕是不考虑的。
    方法:本GL是按照意大利国家指南系统手册中描述的方法开发的。对于每个问题,由Medici内分泌协会(AME)任命的小组已经确定了潜在的相关结果,然后根据它们对治疗选择的影响进行评级。只有分类为“关键”和“重要”的结果在证据的系统评价中被考虑,只有分类为“关键”的那些在建议的制定中被考虑。
    结果:本GL提供了关于药物和神经外科治疗在泌乳素瘤治疗中的作用的建议。我们推荐卡麦角林(Cab)与溴隐亭(Br)作为首选药物治疗,以能够实现临床表现消退的最低有效剂量使用。我们建议为非侵入性PRL分泌腺瘤患者提供药物和手术作为合适的替代一线治疗。无论大小。我们建议将Br作为对Cab不耐受且不适合手术的患者的替代药物。我们建议对患者进行垂体瘤切除术1)从Cab开始的两周内没有任何显着的神经眼科改善,2)耐药或不耐受Cab或其他多巴胺激动剂药物(DA),3)逃避先前DA功效的人,和4)不愿意接受慢性DA治疗的人。我们建议,尽管先前进行过肿瘤切除和进行中的DA,但进行性疾病的患者应由具有垂体疾病专业知识的多学科团队使用包括重复手术在内的多模式方法进行管理。放射治疗,DA,而且可能,使用替莫唑胺。
    结论:目前的GL是针对内分泌学家,神经外科医生,和妇科医生在医院工作,在领土服务或私人执业中,以及全科医生和患者。
    BACKGROUND: This guideline (GL) is aimed at providing a reference for the management of prolactin (PRL)-secreting pituitary adenoma in adults. However, pregnancy is not considered.
    METHODS: This GL has been developed following the methods described in the Manual of the Italian National Guideline System. For each question, the panel appointed by Associazione Medici Endocrinologi (AME) has identified potentially relevant outcomes, which have then been rated for their impact on therapeutic choices. Only outcomes classified as \"critical\" and \"important\" have been considered in the systematic review of evidence and only those classified as \"critical\" have been considered in the formulation of recommendations.
    RESULTS: The present GL provides recommendations regarding the role of pharmacological and neurosurgical treatment in the management of prolactinomas. We recommend cabergoline (Cab) vs. bromocriptine (Br) as the firstchoice pharmacological treatment to be employed at the minimal effective dose capable of achieving the regression of the clinical picture. We suggest that medication and surgery are offered as suitable alternative first-line treatments to patients with non-invasive PRL-secreting adenoma, regardless of size. We suggest Br as an alternative drug in patients who are intolerant to Cab and are not candidates for surgery. We recommend pituitary tumor resection in patients 1) without any significant neuro-ophthalmologic improvement within two weeks from the start of Cab, 2) who are resistant or do not tolerate Cab or other dopamine-agonist drugs (DA), 3) who escape from previous efficacy of DA, and 4) who are unwilling to undergo a chronic DA treatment. We recommend that patients with progressive disease notwithstanding previous tumor resection and ongoing DA should be managed by a multidisciplinary team with specific expertise in pituitary diseases using a multimodal approach that includes repeated surgery, radiotherapy, DA, and possibly, the use of temozolomide.
    CONCLUSIONS: The present GL is directed to endocrinologists, neurosurgeons, and gynecologists working in hospitals, in territorial services or private practice, and to general practitioners and patients.
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  • 文章类型: Journal Article
    生长激素(GH)已使用超过35年,其安全性和有效性已被广泛研究。实验研究表明GH/胰岛素样生长因子1(IGF-I)在致癌作用中的允许作用引起了人们对接受癌症治疗的儿童和成人以及颅内和垂体肿瘤患者GH替代治疗的安全性的担忧。发表了共识声明,以指导儿童和成年癌症幸存者的GH替代决策,在接受颅内和垂体肿瘤治疗的患者以及癌症风险增加的患者中。在欧洲内分泌学会的支持下,生长激素研究协会召开了一个研讨会,代表10个专业学会的55名国际主要舆论领袖应邀参加。这一共识声明利用了:(1)研讨会前编写的一份关键审查文件,(2)五次全会,(3)来自四个突破小组的循证评论,(4)在汇报会议期间进行讨论。从研讨会的程序中审查的当前证据不支持GH替代与原发性肿瘤或癌症复发之间的关联。与主机和肿瘤治疗相关因素相比,GH替代对继发性瘤形成风险的影响较小。在GH缺乏的儿童癌症幸存者中,没有证据表明GH替代与癌症死亡率增加之间存在关联。接受GH替代治疗的垂体瘤或颅咽管瘤患者无需与未接受GH治疗的患者进行不同的治疗或监测。经过仔细的个体风险/收益分析,GH缺乏的成年癌症幸存者在缓解后可能会考虑GH替代。在患有癌症倾向综合征的儿童中,GH治疗通常是禁忌的,但在某些患者中可以谨慎考虑。
    Growth hormone (GH) has been used for over 35 years, and its safety and efficacy has been studied extensively. Experimental studies showing the permissive role of GH/insulin-like growth factor 1 (IGF-I) in carcinogenesis have raised concerns regarding the safety of GH replacement in children and adults who have received treatment for cancer and those with intracranial and pituitary tumours. A consensus statement was produced to guide decision-making on GH replacement in children and adult survivors of cancer, in those treated for intracranial and pituitary tumours and in patients with increased cancer risk. With the support of the European Society of Endocrinology, the Growth Hormone Research Society convened a Workshop, where 55 international key opinion leaders representing 10 professional societies were invited to participate. This consensus statement utilized: (1) a critical review paper produced before the Workshop, (2) five plenary talks, (3) evidence-based comments from four breakout groups, and (4) discussions during report-back sessions. Current evidence reviewed from the proceedings from the Workshop does not support an association between GH replacement and primary tumour or cancer recurrence. The effect of GH replacement on secondary neoplasia risk is minor compared to host- and tumour treatment-related factors. There is no evidence for an association between GH replacement and increased mortality from cancer amongst GH-deficient childhood cancer survivors. Patients with pituitary tumour or craniopharyngioma remnants receiving GH replacement do not need to be treated or monitored differently than those not receiving GH. GH replacement might be considered in GH-deficient adult cancer survivors in remission after careful individual risk/benefit analysis. In children with cancer predisposition syndromes, GH treatment is generally contraindicated but may be considered cautiously in select patients.
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