دورية أكاديمية
Non-functional alpha-cell hyperplasia with glucagon-producing NET: a case report.
| العنوان: | Non-functional alpha-cell hyperplasia with glucagon-producing NET: a case report. |
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| المؤلفون: | Cidade-Rodrigues C; Hospital Padre Américo, Unidade Local de Saúde do Tâmega e Sousa, Penafiel, Portugal., Santos AP; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal.; Research Center of IPO Porto (CI-IPOP), RISE@CI-IPO (Health Research Network), Portuguese Oncology Institute of Porto (IPO Porto), Porto Comprehensive Cancer Centre (P.CCC), Porto, Portugal., Calheiros R; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Santos S; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Matos C; Hospital de Braga, Unidade Local de Saúde de Braga, Braga, Portugal., Moreira AP; Institute for Nuclear Sciences Applied to Health (ICNAS), University of Coimbra, Coimbra, Portugal., Inácio I; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Souteiro P; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Oliveira J; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Jácome M; Department of Pathology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Pereira SS; Unit for Multidisciplinary Research in Biomedicine (UMIB), School of Medicine and Biomedical Sciences (ICBAS), University of Porto, Porto, Portugal.; Laboratory of Integrative and Translocation Research in Population Health (ITR), Porto, Portugal., Henrique R; Research Center of IPO Porto (CI-IPOP), RISE@CI-IPO (Health Research Network), Portuguese Oncology Institute of Porto (IPO Porto), Porto Comprehensive Cancer Centre (P.CCC), Porto, Portugal.; Department of Pathology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal.; Department of Pathology and Molecular Immunology, School of Medicine and Biomedical Sciences (ICBAS), University of Porto, Porto, Portugal., Torres I; Department of Endocrinology, Portuguese Oncology Institute of Porto (IPO Porto), Porto, Portugal., Monteiro MP; Unit for Multidisciplinary Research in Biomedicine (UMIB), School of Medicine and Biomedical Sciences (ICBAS), University of Porto, Porto, Portugal.; Laboratory of Integrative and Translocation Research in Population Health (ITR), Porto, Portugal. |
| المصدر: | Frontiers in endocrinology [Front Endocrinol (Lausanne)] 2024 Sep 06; Vol. 15, pp. 1405835. Date of Electronic Publication: 2024 Sep 06 (Print Publication: 2024). |
| نوع المنشور: | Case Reports; Journal Article |
| اللغة: | English |
| بيانات الدورية: | Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101555782 Publication Model: eCollection Cited Medium: Print ISSN: 1664-2392 (Print) Linking ISSN: 16642392 NLM ISO Abbreviation: Front Endocrinol (Lausanne) Subsets: MEDLINE |
| أسماء مطبوعة: | Original Publication: [Lausanne : Frontiers Research Foundation] |
| مواضيع طبية MeSH: | Hyperplasia*/metabolism , Hyperplasia*/pathology , Glucagon-Secreting Cells*/metabolism , Glucagon-Secreting Cells*/pathology , Glucagon*/metabolism , Neuroendocrine Tumors*/metabolism , Neuroendocrine Tumors*/pathology , Neuroendocrine Tumors*/diagnosis , Pancreatic Neoplasms*/metabolism , Pancreatic Neoplasms*/pathology , Pancreatic Neoplasms*/diagnosis, Humans ; Male ; Aged ; Diabetes Mellitus, Type 2/metabolism ; Diabetes Mellitus, Type 2/complications |
| مستخلص: | Introduction: Alpha-cell hyperplasia (ACH) is a rare pancreatic endocrine condition. Three types of ACH have been described: functional or nonglucagonoma hyperglucagonemic glucagonoma syndrome, reactive or secondary to defective glucagon signaling, and non-functional. Few cases of ACH with concomitant pancreatic neuroendocrine tumors (pNETs) have been reported and its etiology remains poorly understood. A case report of non-functional ACH with glucagon-producing NET is herein presented. Case Report: A 72-year-old male was referred to our institution for a 2 cm single pNET incidentally found during imaging for acute cholecystitis. The patient's past medical history included type 2 diabetes (T2D) diagnosed 12 years earlier, for which he was prescribed metformin, dapagliflozin, and semaglutide. The pNET was clinically and biochemically non-functioning, apart from mildly elevated glucagon 217 pg/ml (<209), and 68 Ga-SSTR PET/CT positive uptake was only found at the pancreatic tail (SUVmax 11.45). The patient underwent a caudal pancreatectomy and the post-operative 68 Ga-SSTR PET/CT was negative. A multifocal well-differentiated NET G1, pT1N0M0R0 (mf) strongly staining for glucagon on a background neuroendocrine alpha-cell hyperplasia with some degree of acinar fibrosis was identified on pathology analysis. Discussion and Conclusion: This case reports the incidental finding of a clinically non-functioning pNET in a patient with T2D and elevated glucagon levels, unexpectedly diagnosed as glucagon-producing NET and ACH. A high level of suspicion was required to conduct the glucagon immunostaining, which is not part of the pathology routine for a clinically non-functioning pNET, and was key for the diagnosis that otherwise would have been missed. This case highlights the need to consider the diagnosis of glucagon-producing pNET on an ACH background even in the absence of glucagonoma syndrome. Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision. (Copyright © 2024 Cidade-Rodrigues, Santos, Calheiros, Santos, Matos, Moreira, Inácio, Souteiro, Oliveira, Jácome, Pereira, Henrique, Torres and Monteiro.) |
| References: | Diabetologia. 2000 Aug;43(8):1012-9. (PMID: 10990079) Neuroendocrinology. 2016;103(2):153-71. (PMID: 26742109) Medicine (Baltimore). 1996 Mar;75(2):53-63. (PMID: 8606627) Am J Pathol. 1998 Jul;153(1):223-31. (PMID: 9665483) Endocrinology. 2004 Jun;145(6):2653-9. (PMID: 15044356) World J Surg. 1984 Aug;8(4):561-74. (PMID: 6207668) J Clin Endocrinol Metab. 2009 Jan;94(1):213-7. (PMID: 18957496) J Am Acad Dermatol. 2011 Aug;65(2):458-459. (PMID: 21763589) Endocrine. 2020 Jun;68(3):518-525. (PMID: 32103407) Endocrinol Nutr. 2012 Nov;59(9):523-30. (PMID: 22951296) J Clin Endocrinol Metab. 2014 Mar;99(3):748-56. (PMID: 24285676) J Diabetes Investig. 2018 Nov;9(6):1270-1282. (PMID: 29570955) J Gastrointest Surg. 1998 Nov-Dec;2(6):533-6. (PMID: 10457311) Diabetes Care. 2013 Jul;36(7):2118-25. (PMID: 23645885) J Clin Invest. 2012 Jan;122(1):4-12. (PMID: 22214853) Pancreas. 2008 May;36(4):428-31. (PMID: 18437091) Diabetes. 2013 Jul;62(7):2595-604. (PMID: 23524641) PLoS One. 2011;6(8):e23397. (PMID: 21853126) Front Pharmacol. 2022 Oct 25;13:925377. (PMID: 36386208) Diabetes Obes Metab. 2020 Apr;22(4):699-704. (PMID: 31750601) Dig Dis Sci. 1991 Jul;36(7):933-42. (PMID: 2070707) Endocr Relat Cancer. 2023 Aug 07;30(9):. (PMID: 37343152) Expert Opin Drug Saf. 2024 Jun;23(6):731-742. (PMID: 37986140) Cancers (Basel). 2024 Apr 23;16(9):. (PMID: 38730578) World J Gastroenterol. 2011 Jan 14;17(2):137-43. (PMID: 21245985) Front Endocrinol (Lausanne). 2022 Jun 16;13:928016. (PMID: 35784565) BMJ. 2016 Feb 17;352:i581. (PMID: 26888382) Med Oncol. 2007;24(3):330-7. (PMID: 17873310) Diabetes Obes Metab. 2021 Sep;23 Suppl 3:5-29. (PMID: 34310013) Endocr Relat Cancer. 2012 Sep 05;19(5):F77-88. (PMID: 22691625) J Hepatobiliary Pancreat Surg. 1998;5(3):312-9. (PMID: 9880781) Diabetologia. 2016 Jul;59(7):1372-1375. (PMID: 27115412) Endocr Rev. 2020 Apr 1;41(2):. (PMID: 31555796) Clin Res Hepatol Gastroenterol. 2012 Jun;36(3):e43-7. (PMID: 22239827) Int J Clin Pharm. 2023 Jun;45(3):689-697. (PMID: 36977858) Am J Med Sci. 2001 May;321(5):306-20. (PMID: 11370794) JAMA Netw Open. 2024 Jan 2;7(1):e2350408. (PMID: 38175642) Am J Surg Pathol. 2006 May;30(5):560-74. (PMID: 16699310) |
| فهرسة مساهمة: | Keywords: alpha-cell; glucagon-producing NET; hyperplasia; neuroendocrine tumors; pancreas |
| المشرفين على المادة: | 9007-92-5 (Glucagon) |
| تواريخ الأحداث: | Date Created: 20240923 Date Completed: 20240923 Latest Revision: 20240924 |
| رمز التحديث: | 20240924 |
| مُعرف محوري في PubMed: | PMC11412808 |
| DOI: | 10.3389/fendo.2024.1405835 |
| PMID: | 39309109 |
| قاعدة البيانات: | MEDLINE |
Full Text Finder | تدمد: | 1664-2392 |
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| DOI: | 10.3389/fendo.2024.1405835 |