Endocrine oncology (Bristol, England)最新文献

{"title":"Systematic review of fluorescence-guided surgery in pituitary neuroendocrine tumours.","authors":"Christopher Dillon Ovenden, Nicholas Candy, James McNeil, Ryan Beerling Dolovac, Mendel Castle-Kirszbaum, Amer Helal, Sunita De Sousa, Alkis Psaltis","doi":"10.1530/EO-25-0037","DOIUrl":"10.1530/EO-25-0037","url":null,"abstract":"<p><strong>Objective: </strong>Fluorescence-guided surgery represents a potential novel technique to improve the extent of tumour resection, minimise resection of normal tissue, and optimise outcomes in pituitary neuroendocrine tumour surgery. We systematically reviewed the available evidence on this topic to define the current role for fluorescence-guided surgery in this setting.</p><p><strong>Design and methods: </strong>A systematic review was conducted in accordance with the PRISMA 2020 guidelines. MEDLINE, EMBASE, and the Cochrane Library were searched on 1st March 2025 for studies investigating the use of exogenous fluorophores or autofluorescence in pituitary surgery. Two authors independently screened studies and extracted data from included studies. Risk of bias assessment was performed using the Newcastle-Ottawa Scale.</p><p><strong>Results: </strong>From 1,604 studies identified, 22 studies involving 297 patients were included. Risk of bias was high in the vast majority of studies. Studies on six exogenous fluorophores were identified (indocyanine green, OTL38, bevacizumab-800CW, chlorin E6 photosensitiser, 5-aminolevulinic acid, and sodium fluorescein), with three studies assessing autofluorescence. Findings of included studies were variable, with small sample sizes and limited outcome reporting. 5-Aminolevulinic acid seems to have little use as a fluorophore in pituitary surgery. The clinical utility of the remaining fluorophores is not yet clearly defined. There are limited data on the utility of autofluorescence in pituitary surgery.</p><p><strong>Conclusion: </strong>At present, there is insufficient evidence to support the routine use of fluorescence-guided surgery in this setting, prompting the need for further research in this area.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250037"},"PeriodicalIF":0.0,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12495904/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145234453","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"⁶⁸Ga-DOTA-TATE PET/CT improves accuracy and guides management in multiple endocrine neoplasia type 1 (MEN-1) patients with suspected duodeno-pancreatic neuroendocrine tumours.","authors":"Kalyan Vamshi Vemulapalli, Kalyan Mansukhbhai Shekhda, Gowri Ratnayake, Gopinath Gnanasegaran, Ann-Marie Quigley, Aimee R Hayes, Bernard Khoo, Dalvinder Mandair, Christos Toumpanakis, Martyn Caplin, Ashley B Grossman, Shaunak Navalkissoor","doi":"10.1530/EO-25-0060","DOIUrl":"10.1530/EO-25-0060","url":null,"abstract":"<p><strong>Purpose: </strong>To evaluate the added benefit and accuracy of ⁶⁸Ga-DOTA-TATE PET/CT scans in detecting duodeno-pancreatic neuroendocrine tumours (dpNETs) compared to conventional cross-sectional imaging with CT or MRI scans in patients with multiple endocrine neoplasia type 1 (MEN-1), and whether the results from the ⁶⁸Ga-DOTA-TATE PET/CT produce a change in management plans for patients with MEN-1 and dpNETs.</p><p><strong>Methods: </strong>A retrospective analysis was performed comparing the initial ⁶⁸Ga-DOTA-TATE PET/CT to the respective contemporary CT or MRI imaging in patients with MEN-1 under the care of a tertiary neuroendocrine centre. Imaging and electronic patient records were analysed to identify treatment plans and the records of multidisciplinary team discussions.</p><p><strong>Results: </strong>In total, 85% (<i>n</i> = 39/46) of patients with MEN-1 had a ⁶⁸Ga-DOTA-TATE PET/CT study in the electronic patient record; 23 of those with duodeno-pancreatic lesions detected also had contemporaneous contrast-enhanced CT scans, while 18 had MRI scans. ⁶⁸Ga-DOTA-TATE PET/CT detected a total of 47 pancreatic lesions compared to 25 on CT, while ⁶⁸Ga-DOTA-TATE PET/CT detected 32 pancreatic lesions compared to 25 on MRI. There were no duodenal lesions detected on conventional CT or MRI, but in comparison to CT and MRI, ⁶⁸Ga-DOTA-TATE PET/CT detected eight and one duodenal lesions respectively. While ⁶⁸Ga-DOTA-TATE PET/CT detected more liver metastases compared to CT (<i>n</i>: 31 vs 21) and similar numbers compared to MRI (<i>n</i>: 11 vs 11), these differences were not statistically significant. As a result of findings on ⁶⁸Ga-DOTA-TATE PET/CT, a change of management was indicated in 69% (<i>n</i> = 27/39) of patients. Of these, 14 patients were offered somatostatin analogues (SSTA), eight patients were offered surgical intervention, three patients were offered peptide receptor radionuclide therapy, and one patient was offered ablation of liver metastases.</p><p><strong>Conclusions: </strong>In patients with MEN-1, ⁶⁸Ga-DOTA-TATE PET/CT was shown to detect a greater number of duodeno-pancreatic lesions compared to conventional cross-sectional CT or MRI imaging. Management plans were changed in most patients following their initial ⁶⁸Ga-DOTA-TATE PET/CT. Therefore, we suggest that somatostatin receptor-targeted PET/CT scans should be an integral part of the investigation of patients with MEN-1 for staging of suspected duodeno-pancreatic NETs.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250060"},"PeriodicalIF":0.0,"publicationDate":"2025-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12449681/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145115086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Efficacy of systemic therapy in metastatic adrenocortical carcinoma: a meta-analysis of prospective clinical trials.","authors":"S Shekar, A Hall, J Pham, M Crumbaker, J Liu, B Talmor, H W Sim, A M Joshua","doi":"10.1530/EO-25-0027","DOIUrl":"10.1530/EO-25-0027","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate pooled prospective trial data investigating efficacy of systemic therapies in metastatic adrenocortical carcinoma (mACC), incorporating subgroup analyses by treatment modality and line, establishing survival and response benchmarks.</p><p><strong>Methods: </strong>Prospective trials of systemic therapy regimens in mACC published from 2010 to July 2023 were included. Primary endpoints were overall survival (OS), progression-free survival (PFS), and objective response rates (ORRs). ORR was logit-transformed and pooled using a random effects model and inverse variance method. Kaplan-Meier curves for PFS and OS were digitised, and summary curves were constructed using a multivariate extension of the DerSimonian-Laird method.</p><p><strong>Results: </strong>Across twenty-four studies, 880 patients were included: 386 received chemotherapy, 169 immunotherapies, 288 targeted therapies, and 37 other therapies. Treatment settings were first-line (383 patients, 44%), second-line or beyond (471, 54%), and not reported (26, 3%). Pooled ORR was 9.0% (95% CI 6.0-13.2) with moderate heterogeneity (<i>I</i> ² = 54.2%, <i>P</i> < 0.01). Subgroup analysis showed ORRs of 2.9% for targeted therapy, 12.3% for chemotherapy, and 15.3% for immunotherapy. Pooled median OS was 9.9 months (95% CI 7.7-11.9) and pooled median PFS 2.6 months (95% CI 1.9-3.6). The 12-month OS rate was 41.6% (95% CI 33.1-51.2) and the 6-month PFS rate was 24.0% (95% CI 15.2-37.8).</p><p><strong>Conclusion: </strong>This analysis establishes contemporary benchmarks for systemic treatments in mACC. While chemotherapy and immunotherapy offer modest survival benefits, the limited effectiveness of targeted therapy highlights the paucity of actionable molecular biomarkers. Future trials should aim to surpass the survival outcomes identified in this analysis.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250027"},"PeriodicalIF":0.0,"publicationDate":"2025-09-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12421987/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145042380","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Diagnostic characteristics, treatment outcomes, and prognostic factors in glucagonomas.","authors":"Eleni Armeni, Alexander Branton, Juliana Porto, Dalvinder Mandair, Aimee R Hayes, Aspasia Manta, Shaunak Navalkissoor, Gopinath Gnanasegaran, Ann-Marie Quigley, Ashley B Grossman, Martyn Caplin, Christos Toumpanakis","doi":"10.1530/EO-24-0083","DOIUrl":"10.1530/EO-24-0083","url":null,"abstract":"<p><strong>Objective: </strong>Glucagonomas are rare islet cell tumours, accounting for 2% of such tumours, with an annual incidence of 0.01-0.1 per million. This study aimed to describe diagnostic characteristics and treatment outcomes in patients with glucagonoma from a major referral centre.</p><p><strong>Design: </strong>A retrospective case series included patients diagnosed with glucagonoma at the ENETS Centre of Excellence, Royal Free Hospital, London, UK.</p><p><strong>Methods: </strong>Electronic patient records were reviewed to document baseline disease characteristics and treatment outcomes. Disease-free survival (DFS), progression-free survival (PFS), and overall survival (OS) were calculated using the Kaplan-Meier method.</p><p><strong>Results: </strong>Twenty patients (75% male, age 56.6 ± 11.6 years, mean Ki-67 index 7.3 ± 7, mean ± SD) were included; 50% had liver metastases at diagnosis. The median OS was 34 months (95% CI: 30.3-37.7). Median OS was 34, 9, and 71 months for patients with liver, lung, and skeletal metastases, respectively. At diagnosis, migratory necrolytic erythema was linked to poorer OS (22 months, 95% CI: 14.3-29.7). Median DFS following surgery was 25 months (95% CI: 3.5-46.5). For inoperable disease, Lutetium-177 (¹⁷⁷Lu)-DOTATATE peptide receptor radionuclide therapy (PRRT) demonstrated efficacy in disease control. Other treatments included somatostatin analogues, chemotherapy, and molecular-targeted agents.</p><p><strong>Conclusion: </strong>Tumour grade, metastases, and NME at diagnosis influence OS in glucagonoma. Surgery is associated with the best PFS as first-line therapy, while ¹⁷⁷Lu-DOTATATE PRRT effectively controls disease progression. Further studies are needed to optimise treatment sequencing for advanced glucagonoma.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e240083"},"PeriodicalIF":0.0,"publicationDate":"2025-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12392752/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144981029","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"FAK inhibition disrupts tumor growth, apoptosis, and transcriptional regulation in GI-NETs.","authors":"Lara Toffoli, Angeliki Ditsiou, Luca Triboli, Victorine Hamm, Eva Moschioni, Francesca D'Este, Teresa Gagliano","doi":"10.1530/EO-25-0052","DOIUrl":"10.1530/EO-25-0052","url":null,"abstract":"<p><strong>Background: </strong>Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms with limited therapeutic options and increasing clinical incidence. Focal adhesion kinase (FAK) has been implicated in oncogenic processes across various tumor types; however, its specific role in GI-NET biology remains inadequately characterized. This study investigates the impact of FAK inhibition on GI-NET cell survival, invasive potential, and gene regulation, with the aim of evaluating FAK as a therapeutic target.</p><p><strong>Methods: </strong>Human GI-NET cell lines (GOT1 and COLO320DM) were treated with Y15, a kinase inhibitor, and PROTAC-FAK (BI-0319), a degrader that abrogates both enzymatic and scaffold functions. siRNA-mediated knockdown of FAK was employed for functional validation. Assays assessing viability and apoptosis were performed in both 2D and 3D culture conditions, while invasion and colony formation were assessed in 2D culture. Western blotting, immunofluorescence, and qRT-PCR were used to evaluate molecular effects. Public transcriptomic datasets were analyzed to assess PTK2 expression across NET subtypes.</p><p><strong>Results: </strong>FAK inhibition reduced cell viability, colony formation, and invasive capacity. PROTAC-FAK, but not Y15, decreased H3K9 acetylation, indicating scaffold-dependent epigenetic modulation. On the other hand, both PROTAC-FAK and Y15 decreased H3K4 methylation levels, further supporting the role of FAK in chromatin regulation. Both compounds suppressed ERK1/2 phosphorylation and modulated RB1 expression, which was further validated by FAK knockdown. In silico analysis revealed elevated PTK2 expression in rectal and small intestinal NETs relative to pancreatic NETs.</p><p><strong>Conclusion: </strong>These findings identify FAK as a regulator of oncogenic and epigenetic pathways in GI-NETs and support its therapeutic targeting, particularly through degradation strategies that inhibit its non-catalytic functions.</p><p><strong>Highlights: </strong>FAK inhibition impairs GI-NET viability, invasion, and colony formation in both 2D and 3D models using kinase (Y15) and PROTAC-based degraders (BI-0319).PROTAC-FAK uniquely reduces H3K9 acetylation, revealing a kinase-independent scaffold role for FAK in epigenetic regulation.esiRNA knockdown of FAK recapitulates pharmacological effects, confirming FAK as a driver of oncogenic features in GI-NET cells.In silico analysis identifies elevated PTK2 expression in rectal and small intestine NETs, with a strong positive correlation to RB1, supporting translational relevance.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250052"},"PeriodicalIF":0.0,"publicationDate":"2025-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12358824/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144884357","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Survival and prognostic differences between appendiceal NETs and goblet cell adenocarcinomas.","authors":"Kim Dijke, José van den Berg, Koert F D Kuhlmann, Sonja Levy, Gerlof D Valk, Margot E T Tesselaar","doi":"10.1530/EO-25-0028","DOIUrl":"10.1530/EO-25-0028","url":null,"abstract":"<p><strong>Objective: </strong>Patients with appendiceal neuroendocrine tumours (aNETs) have an excellent prognosis. Appendiceal goblet cell adenocarcinomas (aGCAs), formerly called goblet carcinoid, show overlapping features with aNETs. Many discrepancies exist between studies regarding prognostication of patients with aNETs. In this study, we aim to identify differences in disease course between aNETs and aGCAs to explain inconsistencies in the literature and identify variables influencing recurrence and survival.</p><p><strong>Methods: </strong>Patients with aNET or aGCA diagnosed between 2000 and 2019 were included. Kaplan-Meier curves were performed in patients with aNET and aGCA independently and in a combined group covering both aNETs and aGCAs to assess progression-free survival (PFS) and disease-specific survival (DSS). Cox regression was used to identify variables influencing PFS and DSS.</p><p><strong>Results: </strong>In total, 122 patients were included: 92 with aNET and 30 with aGCA. Five- and 10-year PFS rates in patients with aNET were both 98%, whereas in patients with aGCA, this was 57% and 30%, respectively. The 5- and 10-year DSS rates for aNETs were 100 and 96%, and for aGCAs, this was 77 and 58%. In the combined group, 5- and 10-year DSS were 92 and 84%, and the presence of peritoneal metastases showed worse DSS (<i>P</i> < 0.001). WHO grade 3 was associated with poorer PFS (HR 18.68, 95% CI (2.24-155.59), <i>P</i> = 0.007) and DSS (HR = 10.21, 95% CI (1.23-85.08), <i>P</i> = 0.032) in aGCAs.</p><p><strong>Conclusion: </strong>aNETs and aGCAs are different entities with a distinct prognosis. Differences in DSS between aNETs and aGCAs indicate previous studies misclassified aggressive tumours as aNETs, which addresses the importance of accurate tumour registration and reckoning with changes in nomenclature.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250028"},"PeriodicalIF":0.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12344360/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144849964","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Epidemiological insights and survival patterns in neuroendocrine neoplasia: a geographic perspective.","authors":"Jessica Mangion, Josanne Vassallo, Mark Gruppetta","doi":"10.1530/EO-25-0039","DOIUrl":"10.1530/EO-25-0039","url":null,"abstract":"<p><p>Neuroendocrine neoplasms, though rare, have shown marked increases in global incidence and prevalence over the past decade, as demonstrated by cancer registry data and studies from specialised tertiary centres. However, it remains unclear whether this represents a true increase in incidence or enhanced detection capabilities across various countries. This review aims to analyse and discuss recently published data on the worldwide epidemiology of gastroenteropancreatic NEN, and bronchopulmonary NEN and explore potential trends and differences in incidence rates between large and small nations according to demographics, primary sites, grade and stage. Following PRISMA guidelines, 59 cohort studies published between 2014 and 2024 were analysed. Findings reveal mixed demographic patterns, with a slight male predominance in overall NEN incidence but site-specific variations influenced by genetic, hormonal, and environmental factors. Median age at diagnosis is 60 years, though appendiceal NENs typically affect younger individuals. Racial disparities were noted, with higher incidence rates among Black populations in the USA, though findings varied across studies. Globally, the incidence of NENs has increased, with the most notable surge reported in the USA between 1973 and 2012. Similar upward trends were observed in Europe, Asia, and Australia, though to different extents. However, variations in data collection methods present significant challenges for cross-national comparisons. The review highlights the need for standardised methodologies and further research to address gaps in understanding NEN epidemiology in smaller nations and underrepresented regions.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250039"},"PeriodicalIF":0.0,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12329783/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144801108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unmasking insulinoma following commencement of somatostatin analogues in malignant neuroendocrine tumours.","authors":"Isuru Gamage, Emma Boehm, Gaurav Ghosh, Grace Kong, Michael Michael, HuiLi Wong, Oliver Piercey, Nirupa Sachithanandan","doi":"10.1530/EO-25-0005","DOIUrl":"10.1530/EO-25-0005","url":null,"abstract":"&lt;p&gt;&lt;strong&gt;Objective: &lt;/strong&gt;Somatostatin analogues (SSA) are used in the management of patients with metastatic gastroenteropancreatic neuroendocrine tumours (GEP-NET) to control hormone secretion and tumour growth. SSA can paradoxically worsen or unmask hypoglycaemia in patients with insulinoma by inhibiting counter-regulatory hormones such as glucagon and growth hormone.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Design and methods: &lt;/strong&gt;We present two cases of SSA use in patients with initially presumed non-functioning GEP-NET unmasking insulinoma. We review the use of SSA in GEP-NET and the management of refractory hypoglycaemia in metastatic insulinoma.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;A 62-year-old female with metastatic grade 2 GEP-NET was commenced on monthly lanreotide 10 weeks after diagnosis. She presented 1 week following the second dose with refractory hypoglycaemia and inappropriate hyperinsulinism, requiring inpatient dextrose infusion. SSA was stopped; however, she remained dextrose dependent despite the addition of diazoxide and dexamethasone. Peptide receptor radionuclide therapy (PRRT) with &lt;sup&gt;177&lt;/sup&gt;Lu-DOTA-Octreotate was given, resulting in resolution of hypoglycaemia after two cycles. The second case is a 57-year-old female with metastatic grade 2 GEP-NET. Four months post commencement of lanreotide, she presented with radiological disease progression and symptomatic hypoglycaemia. A 72 h fast confirmed hyperinsulinaemic hypoglycaemia. SSA was stopped. A trial of diazoxide was not tolerated, and a prednisolone trial was ineffective. The patient underwent inpatient PRRT with euglycaemia achieved shortly afterwards.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions: &lt;/strong&gt;SSA can unmask hypoglycaemia secondary to insulinoma. Detection of new-onset hypoglycaemia requires careful clinical vigilance when commencing SSA in patients with GEP-NET initially presumed to be non-functional. Hypoglycaemia from metastatic insulinoma requires multidisciplinary management incorporating nutritional, medical and oncologic therapy. PRRT can be effective in managing refractory hypoglycaemia.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Learning points: &lt;/strong&gt;SSA use can unmask insulinoma in a NET presumed to be non-functional.SSA can paradoxically worsen hypoglycaemia in insulinoma due to suppression of counter-regulatory hormones.There are currently no biomarkers in routine clinical use to predict which patients will experience a worsening of hypoglycaemia after SSA initiation. Detection of new-onset hypoglycaemia requires clinical vigilance and education of patients to report symptoms early to enable prompt investigation and management.Symptomatic hypoglycaemia in metastatic insulinoma is challenging to manage and requires a multidisciplinary approach considering diet, medical therapy and urgent initiation or escalation of oncologic therapy.PRRT is a safe and effective strategy to achieve hormonal and oncologic control in metastatic insulinoma. Caution should be practised regarding flare of insulin release, a","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250005"},"PeriodicalIF":0.0,"publicationDate":"2025-07-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12281627/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144692615","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A new link between insulinoma and congenital glucose-galactose malabsorption.","authors":"Antonio Prinzi, Jelka Kuiper, Adorée M van der Wiel, Marie-Louise F van Velthuysen, Remco T P van Cruchten, Lodewijk A A Brosens, Wouter W de Herder, Johannes Hofland","doi":"10.1530/EO-25-0030","DOIUrl":"10.1530/EO-25-0030","url":null,"abstract":"<p><p>Congenital glucose-galactose malabsorption (CGGM) is a rare autosomal recessive disorder caused by a biallelic mutation of solute carrier family 5 member 1 (<i>SLC5A1</i>), encoding the sodium-dependent glucose transport-1 (SGLT-1) protein. Patients with CGGM present with neonatal-onset osmotic diarrhea due to impaired intestinal uptake of glucose. Here, we report a case of a 41-year-old female with CGGM who was referred to our clinic for symptoms of hypoglycemia, with the final diagnosis of an insulinoma, which was successfully resected. Whole genome sequencing of tumor DNA revealed chromosomal aberrations, without the presence of driver mutations. Given the unknown long-term sequelae of SGLT-1 loss of function in adulthood, this first case of insulinoma in a CGGM patient potentially uncovers a new phenotype resulting from decades of imbalance in glucose homeostasis. We hypothesize that SGLT-1 might play a role in the plasticity of pancreatic β-cells and suggest mechanisms through which CGGM patients could potentially have a higher risk of developing insulinoma in adulthood.</p><p><strong>Learning points: </strong>This is the first documented case of insulinoma in a patient with CGGM, suggesting a potential new link between glucose absorption disorders and pancreatic neuroendocrine tumors.The loss-of-function mutation in SGLT-1 due to an SLC5A1 gene mutation may impact pancreatic β-cell plasticity, potentially contributing to insulinoma development through altered glucose homeostasis.The patient's long-term high-fat, low-carbohydrate diet may have played a role in β-cell stimulation via increased levels of GLP-1 and GIP, both of which promote β-cell proliferation and survival.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250030"},"PeriodicalIF":0.0,"publicationDate":"2025-07-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12257603/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144638842","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Prevalence of growth hormone deficiency in brain tumor survivors: a systematic review and meta-analysis.","authors":"Tatiana Tselovalnikova, Ben Ponvilawan, Maria G Pavlova, Cynthia Flanagan, Sunpreet S Rakhra, Betty M Drees","doi":"10.1530/EO-25-0025","DOIUrl":"10.1530/EO-25-0025","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the prevalence of growth hormone deficiency in patients who underwent cranial irradiation for brain tumors.</p><p><strong>Methods: </strong>Ovid Medline and Embase databases were used for review. Eligible studies were observational studies with brain tumor survivors who had growth hormone function evaluated after treatment at age ≥18 years. Patient data on disease prevalence were pooled using the random-effect, generic inverse variance method. The presence of publication bias was determined by Egger's test. Mann-Whitney U test, univariate and multivariate linear regression were used to determine the association and effect of covariates and growth hormone peak level.</p><p><strong>Results: </strong>After screening 3,355 relevant articles, seven studies were included. The pooled result showed that out of 3,489 patients who received radiation for brain tumors, regardless of age at the time of treatment, 50% developed growth hormone deficiency (95% CI 40-60%, <i>I</i> ² = 83%). Subgroup analysis based on the growth hormone peak level did not show differences between different cutoffs. Univariate linear regression using data from 27 patients showed that age at radiation and the time duration between radiation and the stimulation test (<i>P</i> = 0.03 and 0.002, respectively), but not radiation dose or sex, were significantly correlated with growth hormone peak level. After multivariate adjustment, only the time duration between radiation and the stimulation test was associated with decreased growth hormone peak level (<i>P</i> = 0.04).</p><p><strong>Conclusions: </strong>Half of brain tumor survivors develop growth hormone deficiency. A longer duration of follow-up is associated with higher risks of growth hormone deficiency. Lifelong follow-up is essential.</p>","PeriodicalId":72907,"journal":{"name":"Endocrine oncology (Bristol, England)","volume":"5 1","pages":"e250025"},"PeriodicalIF":0.0,"publicationDate":"2025-07-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12243098/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144610425","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}