Neuroendocrinology最新文献

{"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":"Unveiling the Prognostic Role of Synaptophysin in Conventional Colorectal Carcinomas.","authors":"Giovanna Sabella, Giovanni Centonze, Vincenzo Lagano, Andrea Scardino, Filiberto Belli, Giovanna Garzone, Carlotta Pardo, Daniela Galbiati, Sara Pusceddu, Alessandro Mangogna, Valentina Angerilli, Matteo Fassan, Andrea Vingiani, Luca Agnelli, Giancarlo Pruneri, Stefania Gobba, Silvia Uccella, Stefano La Rosa, Fausto Sessa, Carlo Capella, Massimo Milione","doi":"10.1159/000545979","DOIUrl":"10.1159/000545979","url":null,"abstract":"<p><strong>Introduction: </strong>Although neuroendocrine differentiation in colorectal carcinomas (CRCs) has been extensively reported, the biological behavior of adenocarcinomas expressing synaptophysin (Syn) but lacking typical neuroendocrine morphology remains unclear.</p><p><strong>Methods: </strong>We tested 663 conventional CRCs with non-neuroendocrine morphology for Syn expression and correlated the results with clinicopathological and molecular characteristics as well as patient survival (overall survival [OS] and disease-free survival [DFS]). The survival characteristics of Syn expression group were compared to those of conventional CRCs and subsequently to those of 14 minENs.</p><p><strong>Results: </strong>Syn immunohistochemical expression ≥30% was confirmed in 27 (4.1%) patients and correlated with right colon site, grade 2, marked intratumoral lymphocyte infiltrate, and BRAF p.V600E mutation. At univariate analysis, variables associated with poor OS were 10-year increase in age (p = 0.001), stage III-IV (p = 0.001), Syn ≥30% (p = 0.001), infiltrative growth (p = 0.04), and residual tumor R1-2 (p = 0.03). At multivariable analysis, Syn expression in ≥30% of gland-forming tumor cells emerged as an independent negative prognostic factor for both OS and DFS. Moreover, 10-year increase in age, stage III-IV, and Syn ≥30% (p < 0.001) were associated with poor OS and marked peritumoral lymphocyte infiltrate with longer OS (p = 0.02). Comparable results were obtained according to DFS; in addition, right colon site (p = 0.04) was associated with longer DFS while KRAS mutation (p = 0.04) was associated with poor DFS at univariate analysis. MiNEN patients showed a shorter DFS than all conventional adenocarcinomas with or without Syn expression in univariate analyses (p < 0.001).</p><p><strong>Conclusions: </strong>Among conventional CRCs, we provided evidence that Syn expression is associated with worse OS and DFS and contributes to predicting clinical outcome. Future studies should explore the molecular mechanisms underlying the acquisition of the neuroendocrine phenotype to identify new targeted treatment strategies.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-16"},"PeriodicalIF":3.2,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12233971/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144034408","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":"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":"Existence of Coexpressive Role of Kisspeptin and Insulin-2 in the Regulation of Luteinizing Hormone in Chronic Stress-Induced Polycystic Ovarian Syndrome-Like Phenotype in Rattus norvegicus.","authors":"Nitin K Rajashekara, Bindu Jayashankaraswamy, Raghu Nataraj","doi":"10.1159/000546126","DOIUrl":"10.1159/000546126","url":null,"abstract":"<p><strong>Background: </strong>Polycystic ovary syndrome (PCOS) is an ill manifestation of the normal ovarian function that obstructs folliculogenesis. Clinically, patients diagnosed with PCOS possess chronic psychological distress with the downregulated hypothalamus-pituitary-gonadal (HPG) axis under the influence of cortisol, but, in contrast, studies done elsewhere have demonstrated an increased hypothalamus-pituitary activity under the PCOS condition. This contradiction has led to several independent research studies assessing the role of metastatic suppressor genes Kisspeptin (KiSS1) and Insulin (INS2) in regulating LH by acting upon GnRH. The current study demonstrates the coexpressive role of KiSS1 and INS2 in regulating LH and monitoring ovarian health.</p><p><strong>Methodology: </strong>PCOS-like features were induced in the rats by a chronic stress regime, and the parameters were established. Another stress group of animals was dosed with 60 mg/kg body weight of ketoconazole before the stress exposure, and the parameters of the study were estimated and established.</p><p><strong>Results: </strong>The current study has observed that upon chronic stress exposure, the animals have exhibited all the features of PCOS, like hyperandrogenism, cystic follicles with dysregulated estrous cyclicity, an elevated LH, and decreased plasma insulin levels. As hypothesized, a 7-fold increase of KiSS1 expression and a 2-fold increase of INS-2 expressions have been observed in the stress group animals, unlike the corticosterone inhibitor group of animals which have exhibited a controlled phenotype.</p><p><strong>Conclusion: </strong>The obtained relative fold changes in the gene expression level of both KiSS1 and INS2 reveal the association of stress with the pathology of PCOS via neuroendocrine regulation. The study has demonstrated the existence of a putative temporal coupling activity of KiSS1-INS2 expression-driven elevated LH in preclinical Rattus norvegicus models.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-15"},"PeriodicalIF":3.2,"publicationDate":"2025-04-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12158408/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144021006","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":"Indirect Effect Model with Surge Function for Describing Melatonin Circadian Rhythm: Quantitative Comparison and Application between Normal Sleepers and Patients with Delayed Sleep-Wake Phase Disorder.","authors":"Zhanhui Lv, Zimo Zhang, Lu Wang, Sufeng Zhou, Jianguo Sun, Xiuqin Wang, Feng Shao","doi":"10.1159/000546125","DOIUrl":"10.1159/000546125","url":null,"abstract":"<p><strong>Introduction: </strong>This study investigates the circadian rhythm of melatonin in normal sleepers and delayed sleep-wake phase disorder (DSWPD) patients using quantitative pharmacology methods to better understand sleep disorders and their underlying mechanisms.</p><p><strong>Methods: </strong>We developed an indirect effect model incorporating a surge function using data from 10 normal sleepers and 26 DSWPD patients. Model accuracy and stability were evaluated using diagnostic plots, visual predictive check, and bootstrap. Monte Carlo simulations (n = 1,000) quantitatively compared melatonin circadian rhythm characteristics between normal sleepers and DSWPD patients. Finally, Bayesian estimation utilizing external data from 3 normal sleepers and 3 DSWPD patients predicted melatonin concentration at different time points and the dim light melatonin onset (DLMO).</p><p><strong>Results: </strong>An indirect effect model incorporating a surge function effectively described the circadian rhythm of endogenous melatonin. The model estimates a population typical value (relative standard error %) of amplitude, 7.95 (15.47%); peak time, 23:59 (4.13%); peak width, 4.12 (5.78%); elimination rate constant, 1.23 h-1 (21.82%); baseline melatonin concentration, 3.21 pg/mL (23.27%). Monte Carlo simulation revealed that DSWPD patients exhibited approximately 7-h delay in DLMO, similar melatonin elimination rate constants, and a significantly lower melatonin production rate constants compared to normal sleepers. Bayesian estimation of the melatonin circadian characteristics and DLMO in DSWPD patients closely matched actual data, with model-estimated DLMO exhibiting an error margin within ±10%.</p><p><strong>Conclusion: </strong>Compared to normal sleepers, DSWPD patients exhibited a reduced rate of melatonin production, similar rate of melatonin elimination, and delayed DLMO, highlighting notable circadian melatonin profile alterations. The final model employed Bayesian feedback to estimate melatonin circadian rhythm characteristics and DLMO in DSWPD patients using sparsely sampled data.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-14"},"PeriodicalIF":3.2,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12180775/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144045024","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":"Upregulation of SSTR2 Expression and Radioligand Binding of [18F]SiTATE in Neuroendocrine Tumour Cells with Combined Inhibition of Class I HDACs and LSD1.","authors":"Christoph J Auernhammer, Kathrin Zitzmann, Simon Lindner, Harun Ilhan, Peter Bartenstein, Umberto Maccio, Michael Orth, Lea Peischer, Julian Maurer, Gerald Spoettl, Katharina Wang, Christine Spitzweg, Alessa Fischer, Constanze Hantel, Ashley B Grossman, Felix Beuschlein, Karel Pacak, Svenja Nölting","doi":"10.1159/000545073","DOIUrl":"10.1159/000545073","url":null,"abstract":"<p><strong>Introduction: </strong>Peptide receptor radionuclide therapy (PRRT) is a highly effective, targeted treatment option in advanced neuroendocrine tumours (NETs). However, NET patients expressing low levels of Somatostatin receptor type (SSTR) 2 do not benefit from this powerful tool. Recently, several preclinical studies have revealed that histone deacetylase (HDAC) inhibitors can upregulate the expression of SSTR2 and enhance somatostatin ligand binding to tumour cells. In this preclinical study, we explored the effects of single and combined treatment of NET cells with the class I HDAC inhibitor entinostat and the lysine-specific demethylase 1 (LSD1) inhibitor CC-90011, on cell viability, SSTR2 expression and radioligand binding.</p><p><strong>Methods: </strong>The human NET cell lines BON1, NCI-H727, and QGP1 were treated with entinostat, CC-90011 or a combination of both. Cell viability was measured with a cell viability assay. SSTR2 expression was assessed by quantitative PCR, Western blot analysis, and immunohistochemistry. [18F]SiTATE uptake was investigated by a radioligand binding assay.</p><p><strong>Results: </strong>Treatment of NET cells with entinostat, CC-90011, and especially the combination of both reduced tumour cell viability and strongly induced SSTR2 expression resulting in potently enhanced radioligand binding of [18F]SiTATE.</p><p><strong>Conclusion: </strong>Combined inhibition of class I HDACs and LSD1 potently increases SSTR2 expression and consequently radioligand binding and might thus be a putative strategy to improve the outcome of PRRT therapy in patients with NETs.</p>","PeriodicalId":19117,"journal":{"name":"Neuroendocrinology","volume":" ","pages":"1-14"},"PeriodicalIF":3.2,"publicationDate":"2025-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12215164/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143795853","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":"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}