Neuroendocrinology最新文献

{"title":"Prognosis of NETs: Has There Been Improvement over the Last 30 Years?","authors":"Dominique Clement, Debashis Sarker, Aviva Frydman, Andrzej Rak, Vina Soran, David C Llewellyn, Raj Srirajaskanthan","doi":"10.1159/000546613","DOIUrl":"10.1159/000546613","url":null,"abstract":"<p><p>Neuroendocrine tumours (NETs) are uncommon tumours, initially described as \"Carzinoides\" over hundred years ago and had since then multiple changes in terminology and difference in consideration of benign or malignant tumours. There have been multiple subclassifications and definitions made by the World Health Organisation (WHO). Multiple studies suggest an increase in incidence and prevalence. There are three types of sources of information for these studies; national databases, regional databases or single-centre studies. These different sources of data describe small or larger cohorts of patients with NETs, including risks of bias and concerns regarding accuracy of data. The studies aim to describe the prognosis of patients with NETs, using outcomes as overall survival (OS), progression-free survival and relative survival rate. There is a heterogeneity of studies including different patient populations, different study periods, different definitions, and different outcomes of prognosis it is difficult to compare studies.This review aims to describe how the prognosis changed in the past 30 years for patients with NETs taken into account changes in treatment. During the past 3 decades, new treatments including targeting somatostatin receptors with somatostatin analogues or peptide receptor radionucleide therapy, systemic anti-cancer treatments with Sunitinib, Everolimus, and Cabozantinib were developed. In this review, the treatments and prognosis between 1990 and 2000 are described. Subsequently per decade 2000-2010, 2010-2020, and 2020-currently, new treatments and up to date studies regarding the prognosis are reviewed. The aim of this study was to explain changes in prognosis of patients with NETs.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-13"},"PeriodicalIF":2.8,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12306953/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144199674","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}

{"title":"Craniopharyngioma, Rathke Cleft Cyst, and Empty Sella Natural History and Endocrine Outcome in Pediatric Age: A Single Tertiary Center Experience over the 2000-2024 Period.","authors":"Gerdi Tuli, Jessica Munarin","doi":"10.1159/000546493","DOIUrl":"10.1159/000546493","url":null,"abstract":"<p><strong>Introduction: </strong>Craniopharyngioma (CP), Rathke cleft cyst (RCC), and empty sella (ES) are clinical conditions that may lead to endocrine disorders.</p><p><strong>Methods: </strong>Clinical data of all pediatric patients aged 0-18 years with diagnosis of CP, RCC, or ES, referred to our department over the period 2000-2024 were analyzed.</p><p><strong>Results: </strong>Data of 13 subjects with CP, 35 subjects with RCC, and 32 patients with ES were analyzed. Mean age at diagnosis was 6.6 ± 1.2 years in the CP group, 8.45 ± 3.2 years in the RCC group, and 10.55 ± 2.1 years in the ES group (p = 0.03). Prior endocrine disorder was the reason for requesting MRI in 27/80 patients: 1/13 of patients with CP, 16/35 of those with RCC, and 10/32 of patients with ES (p = 0.04). Among RCC patients, higher age and RCC size were observed in subjects with endocrine disorders (p = 0.04). Similar trend was observed in patients with ES, with significantly higher age in those with endocrine disorders compared to those without (p = 0.04). During follow-up, endocrine disorders were diagnosed in all patients with CP (13/13; 100%), in 5 out of 20 patients with RCC (25%), and in none of the patients with ES.</p><p><strong>Conclusion: </strong>CP, RCC, and ES need baseline and over a prolonged period of time endocrinological and neuroradiological follow-up, in order to detect promptly endocrine defects and sellar/suprasellar region alterations, and multidisciplinary follow-up in tertiary centers is mandatory.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-6"},"PeriodicalIF":3.2,"publicationDate":"2025-05-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144102275","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}

{"title":"Impact of Sex and Deprivation on Neuroendocrine Tumour Survival: Challenges of Heterogeneous Data.","authors":"Ailbhe Lawlor, Harriet Wylde, Marie Line El Asmar, Benjamin Easton White, John Ramage, Mieke Van Hemelrijck, Beth Russell","doi":"10.1159/000546128","DOIUrl":"10.1159/000546128","url":null,"abstract":"<p><strong>Background: </strong>For decades, the incidence of neuroendocrine tumours (NETs) has been steadily increasing. Existing research suggests that patient sociodemographic characteristics, such as sex and deprivation, may play a role in who survives a NET diagnosis. This systematic review identifies the available evidence assessing the impact of sex and deprivation on the prognosis of patients diagnosed with NETs.</p><p><strong>Summary: </strong>Using protocol-driven search terms, Embase and Ovid were searched in July 2024. The search identified 2,041 unique citations, of which 66 articles were subsequently included. Findings were reported by geographical location. Included studies indicate a female survival advantage in North America and England, but the impact of sex on NET prognosis in wider European and Asian countries is less clear. The impact of deprivation on NET prognosis was assessed in North America, with one study conducted in Europe.</p><p><strong>Key messages: </strong>Significant data heterogeneity across studies poses challenges to comparability between studies and hinders statistical analyses of these data. In North America and England, females diagnosed with NETs tend to survive longer than males. Existing single-centre studies do not provide conclusive evidence on the impact of sex on NET survival in Asian countries, and a greater number of population-based studies are needed. Future research should also focus on addressing heterogeneity across NET research to allow for more robust evidence synthesis, providing increased accuracy and generalisability of study results.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-24"},"PeriodicalIF":3.2,"publicationDate":"2025-04-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12176355/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144035983","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}

{"title":"Gender Perspective in Lung Neuroendocrine Tumors: A Critical Review.","authors":"Anna La Salvia, Roberta Modica, Francesca Spada, Roberta Elisa Rossi","doi":"10.1159/000546081","DOIUrl":"10.1159/000546081","url":null,"abstract":"<p><strong>Background: </strong>The role of gender has gained attention in oncology. In the setting of lung neuroendocrine tumors (L-NETs), the existence of differences between male and females has been suggested, but no clear-cut data are available. We aimed to provide a critical analysis of the existing literature regarding sex roles in L-NETs.</p><p><strong>Methods: </strong>We performed an extensive search of the available literature to provide a critical narrative review focused on key topics such as epidemiology, histopathological and molecular features, functioning syndromes, prognosis, and response/toxicity to treatments in L-NETs according to sex.</p><p><strong>Results: </strong>Female patients are more likely to have an L-NET than males. The reasons underlying these gender differences are still unclear; a biologic mechanism for the sex difference is possible, through a role of hormones in regulating gene expression and promoting neuroendocrine cell proliferation. A difference in immunohistochemical biomarkers has been found; thyroid transcription factor-1 (TTF-1) expression appears to be associated with female gender; at the molecular level, in the majority of studies, L-NET mutational profile is not stratified for sex. In terms of prognosis, a correlation between male gender and a more aggressive disease has been found. Patient's gender has been recognized as a key modulator in the response/resistance to anticancer treatments; however, for L-NETs, the available data regarding the activity of different treatments and their toxicities are scarce, as in clinical trials designed for L-NETs, a stratified evaluation of drugs' activity according to patients' sex is largely missing.</p><p><strong>Conclusions: </strong>There is emerging evidence suggesting a gender role in L-NETs; however, further studies are needed to better understand the pathogenesis of these tumors and to plan tailored treatments. Graphical Abstract: for Graphical Abstract, see <ext-link ext-link-type=\"doi\" xlink:href=\"https://doi.org/10.1159/000546081\" xmlns:xlink=\"http://www.w3.org/1999/xlink\">https://doi.org/10.1159/000546081</ext-link>.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-8"},"PeriodicalIF":3.2,"publicationDate":"2025-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144040366","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}

{"title":"Clinical and Genetic Factors Associated with Neuroendocrine Neoplasms: A UK Biobank Study.","authors":"Harry David Green, Marie Line El-Asmar, Brian Rous, Gareth Hawkes, Maria Trinidad Moreno-Montilla, Christina Thirlwell, John Ramage","doi":"10.1159/000545114","DOIUrl":"10.1159/000545114","url":null,"abstract":"<p><strong>Introduction: </strong>Incidence of neuroendocrine neoplasms (NENs) is rising globally, yet clinical and genetic factors remain poorly understood. Evidence for the role of obesity is conflicted, and studies on prospectively collected data are sparse. We aimed to identify clinical and germline genetic risk factors associated with NEN in the UK Biobank.</p><p><strong>Methods: </strong>Cases of NEN were identified in the UK Biobank's cancer registry data (N∼500,000). Using a combination of ICD-O3 codes for cancer site and histology, NEN cases were stratified into neuroendocrine tumour (NET), neuroendocrine carcinoma (NEC), and small/large cell lung cancer (SLCLC). A Cox proportional hazards model was used to test for an association between clinical phenotypes and increased NEN risk, and a gene burden test in Regenie was used to test for causal variants in the exome sequencing data.</p><p><strong>Results: </strong>We identified 704 NET, 340 NEC, and 550 SLCLC cases. Obesity (BMI or waist-hip ratio) and lower cholesterol (LDL, HDL, or total) had a significantly significant association with NEN risk; however, the effect size was marginal. Smoking and HbA1c were associated only with SLCLC. Air pollution was not significantly associated when adjustment was made for socio-economic status. We replicated a known germline association between loss of function variants in MEN-1 and NEC, but did not detect any novel association in exome variants.</p><p><strong>Conclusion: </strong>This is the first large prospective population-based study to identify potential clinical and genetic risk factors for NEN and define a novel phenotype in the UK Biobank. More research is needed to establish whether these relationships are causal. The exome study was underpowered, and future work in this area should focus on meta-analysing multiple large datasets.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-5"},"PeriodicalIF":3.2,"publicationDate":"2025-03-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143625348","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}

{"title":"A Review for the Clinician: Classifications, Genetics, and Treatment for Neuroendocrine Neoplasms of the Thymus (Thymic Carcinoids).","authors":"Matthias Lang, Chrysanthi Anamaterou, Isabelle Mohr, Mária Černá, Manuel Röhrich, Christine Tjaden","doi":"10.1159/000544982","DOIUrl":"10.1159/000544982","url":null,"abstract":"<p><strong>Background: </strong>Thymic carcinoids or neuroendocrine neoplasms (t-NEN) are a rare entity with a dismal prognosis. About 25% of the tumors are related to multiple endocrine neoplasia type I (MEN-1), where they contribute significantly to mortality. The tumors are classified according to the WHO classification, TNM classification and Masaoka-Koga staging system, although none of the classifications have been developed for t-NEN. A recently proposed t-NEN specific morphomolecular classification is based on copy number instability scores. Its role is yet to be defined. The prognosis depends on resectability, histological features, metastasis, the amount of copy number instabilities and mitotic activity.</p><p><strong>Summary: </strong>No study-based therapies exist. The mainstay of therapy is surgical resection as it is associated with significantly improved long-term survival. Based on published cases and small series, for non-resectable and recurring disease, platinum-based chemotherapies are preferred in neuroendocrine carcinoma, while everolimus and temozolomide are recommended in thymic neuroendocrine tumors.</p><p><strong>Key messages: </strong>This review covers current classification systems and the knowledge of genetic disorders and medical therapies.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-12"},"PeriodicalIF":3.2,"publicationDate":"2025-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143605378","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}

{"title":"Are Neuroendocrine Neoplasms No Longer a Rare Cancer?","authors":"Catherine Bouvier Ellis, Nicola Jervis","doi":"10.1159/000544984","DOIUrl":"10.1159/000544984","url":null,"abstract":"<p><strong>Background: </strong>Neuroendocrine neoplasms (NENs) are consistently referred to as a \"relatively\" rare heterogenous group of \"tumours\" with variability in their disease course and outcomes. However, there is a lack of consensus on (a) the group membership, that is, a lack of consistency in which \"subtypes\" of NEN are included in the group; (b) whether they should continue to be seen as a \"heterogenous group,\" or as separate entities; and (c) whether the term and current definitions of \"rare\" accurately reflects the true patient population and healthcare requirement.</p><p><strong>Summary: </strong>This opinion article explores the concept of rare, as applied to NENs: the significance of a rare cancer label and what this means for awareness, healthcare provision and, tangentially, those diagnosed. It briefly explores rare cancer definitions, including incidence thresholds and interpretation of definition as demonstrated in the variability in what subtypes are included in databanks or registries, and it also asks whether the currently utilised rare cancer definitions reflect an accurate representation of the true disease burden and fully inform disease-appropriate healthcare planning and provision.</p><p><strong>Key messages: </strong>The current definition of \"rare cancer\" based on incidence alone fails to reflect the true disease burden of NENs and is therefore inadequate, to fully inform healthcare policy, planning and provision for this patient population. This requires either a revision in definition or an alteration in how and what decision-makers utilise and include in their deliberations when assessing and planning service provision.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-5"},"PeriodicalIF":3.2,"publicationDate":"2025-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143586406","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}

{"title":"Factors Influencing Costs of Cancer Care for Patients with Neuroendocrine Neoplasms.","authors":"Martin O Weickert","doi":"10.1159/000544050","DOIUrl":"10.1159/000544050","url":null,"abstract":"<p><strong>Background: </strong>Neuroendocrine neoplasms (NENs) are comparatively rare tumours. However, prevalence is increasing steeply, related to rising incidence, earlier detection, and prolonged survival in many cases of metastatic NENs, with implications on healthcare resources.</p><p><strong>Summary: </strong>This commentary/narrative review extracts the relatively scare, available literature related to costs of NEN cancer care, which is mainly based on studies performed in the USA. Key, now implemented or evolving NEN-related treatment options over the last 15 years, is summarised. The commentary further highlights in part preventable aspects that can further contribute to cost pressure in NEN cancer care, including issues related to inappropriate use of available diagnostic tools, and not considering differential diagnoses when assessing people with suspected carcinoid syndrome - with these risks being minimised with access to centres with multi-speciality expertise in the management of people with NENs. Issues observed in people with exocrine and/or endocrine pancreatic deficiencies caused by a NEN or treatment of the NEN are mentioned, as well as some specific aspects related to diagnostics involving 68Ga PET-CT scans and treatment with Lutetium peptide-receptor radionuclide therapy (Lu-PRRT).</p><p><strong>Key messages: </strong>This commentary summarises factors influencing cost of NEN cancer care, and highlights in part preventable issues mostly related to delayed involvement of a NEN multidisciplinary team, observed in a UK NEN referral centre (ENETS Centre of Excellence certified since 2015) over the last 15 years, resulting in suboptimal management of people with NENs and ultimately adding to cost pressure.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-22"},"PeriodicalIF":3.2,"publicationDate":"2025-02-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143409434","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}

{"title":"Cell Signaling in the Circadian Pacemaker: New Insights from in vivo Imaging.","authors":"Xavier Bonnefont","doi":"10.1159/000539344","DOIUrl":"10.1159/000539344","url":null,"abstract":"<p><strong>Background: </strong>\"One for all, and all for one,\" the famous rallying cry of the Three Musketeers, in Alexandre Dumas's popular novel, certainly applies to the 20,000 cells composing the suprachiasmatic nuclei (SCN). These cells work together to form the central clock that coordinates body rhythms in tune with the day-night cycle. Like virtually every body cell, individual SCN cells exhibit autonomous circadian oscillations, but this rhythmicity only reaches a high level of precision and robustness when the cells are coupled with their neighbors. Therefore, understanding the functional network organization of SCN cells beyond their core rhythmicity is an important issue in circadian biology.</p><p><strong>Summary: </strong>The present review summarizes the main results from our recent study demonstrating the feasibility of recording SCN cells in freely moving mice and the significance of variations in intracellular calcium over several timescales.</p><p><strong>Key message: </strong>We discuss how in vivo imaging at the cell level will be pivotal to interrogate the mammalian master clock, in an integrated context that preserves the SCN network organization, with intact inputs and outputs.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"103-110"},"PeriodicalIF":3.2,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140958594","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}

{"title":"The Hypothalamus and Pituitary Gland Regulate Reproduction and Are Involved in the Development of Polycystic Ovary Syndrome.","authors":"Bin-Yang Long, Xipeng Liao, Xin Liang","doi":"10.1159/000543877","DOIUrl":"10.1159/000543877","url":null,"abstract":"<p><strong>Background: </strong>Polycystic ovary syndrome (PCOS) is a complex condition with unclear mechanisms, posing a challenge for prevention and treatment of PCOS. The role of the hypothalamus and pituitary gland in regulating female reproduction is critical. Abnormalities in the hypothalamus and pituitary can impair reproductive function. It is important to study hypothalamic and pituitary changes in patients with PCOS.</p><p><strong>Summary: </strong>This article reviews articles on the hypothalamus and PCOS with the goal of summarizing what abnormalities of the hypothalamic-pituitary-ovarian axis are present in patients with PCOS and to clarify the pathogenesis of PCOS. We find that the mechanisms by which the hypothalamus and pituitary regulate reproduction in girls are complex and are associated with altered sex hormone levels, obesity, and insulin resistance. Different animal models of PCOS are characterized by different alterations in the hypothalamus and pituitary and respond differently to different treatments, which correspond to the complex pathogenesis of patients with PCOS.</p><p><strong>Key messages: </strong>Arcuate nucleus (ARC) is associated with luteinizing hormone (LH) surges. Suprachiasmatic nucleus, ARC, and RP3V are associated with LH surges. Animal models of PCOS have different characteristics.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"315-334"},"PeriodicalIF":2.8,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143080305","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}