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      Malignant Insulinoma Arising From Nonfunctioning Pancreatic Neuroendocrine Tumor

      case-report

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          ABSTRACT

          Pancreatic neuroendocrine tumors are rare neoplasms characterized into nonfunctioning (NF-PNET) and functioning (F-PNET) subtypes. F-PNETs typically involve overt symptoms related to excessive hormone secretion but may rarely present first as NF-PNETs with delayed transformation. We present a patient with known NF-PNET with liver metastases who developed hypoglycemia 2 years after initial diagnosis due to malignant insulinoma. Hypoglycemia was refractory to continuous dextrose but improved temporarily after diazoxide and hepatic artery embolization. Malignant insulinomas are usually metastatic at presentation and portend poor prognosis. Hypoglycemia may be medically managed with steroids, somatostatin analogues, and diazoxide, along with therapies to reduce tumor burden.

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          Most cited references22

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          Exploring the rising incidence of neuroendocrine tumors: a population-based analysis of epidemiology, metastatic presentation, and outcomes.

          An increased incidence of neuroendocrine tumors (NETs) has been reported worldwide, but the reasons underlying this rise have not been identified. By assessing patterns of metastatic presentation, this study sought to examine the epidemiologic characteristics of NETs and the contribution of early-stage detection to the rising incidence.
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            Multiple and Secondary Hormone Secretion in Patients With Metastatic Pancreatic Neuroendocrine Tumours.

            As a group, neuroendocrine tumors (NETs) secrete many different peptide hormones, yet heretofore each NET patient is typically thought to produce at most one hormone that causes a distinct hormonal syndrome. A minority of patients have multiple hormones at diagnosis and may also develop secondary hormone secretion at a later stage.
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              Adult Pituitary Stem Cells: From Pituitary Plasticity to Adenoma Development

              The pituitary needs high plasticity of the hormone-producing cell compartment to generate the continuously changing hormonal signals that govern the key physiological processes it is involved in, as well as homeostatic cell turnover. However, the underlying mechanisms are still poorly understood. It was proposed that adult stem cells direct the generation of newborn cells with a hormonal phenotype according to the physiological requirements. However, only in recent years adult pituitary stem cells have begun to be phenotypically characterized in several studies that identified multiple stem/progenitor cell candidates. Also considering the incompletely defined features of this cell subpopulation, some discrepancies among the different reports are clearly apparent and long-term self-renewal remains to be unequivocally demonstrated. Here, all the recently published evidence is analyzed, trying, when possible, to reconcile the results of the different studies. Finally, with the perspective of shedding light on pituitary tumorigenesis and the development of potentially new pharmacological approaches directed against these cells, very recent evidence on the presence of putative cancer stem cells in human pituitary adenomas is discussed.
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                Author and article information

                Contributors
                Journal
                ACG Case Rep J
                ACG Case Rep J
                ACGCRJ
                AC9
                ACG Case Reports Journal
                Wolters Kluwer (Maryland, MD )
                2326-3253
                January 2023
                27 January 2023
                : 10
                : 1
                : e00954
                Affiliations
                [1 ]Department of Internal Medicine, Baylor College of Medicine, Houston, TX
                [2 ]Section of Endocrinology, Diabetes, and Metabolism, Department of Internal Medicine, Baylor College of Medicine, Houston, TX
                [3 ]Section of Hematology and Oncology, Department of Internal Medicine, Baylor College of Medicine, Houston, TX
                Author notes
                Correspondence: Ritodhi Chatterjee, MD ( Ritodhi.chatterjee@ 123456bcm.edu ).
                Author information
                https://orcid.org/0000-0002-3976-9445
                Article
                ACGCR-22-0510 00022
                10.14309/crj.0000000000000954
                10914231
                38445216
                96446d3c-11f1-47ac-a0da-38cbb4bd797b
                © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

                History
                : 31 July 2022
                : 09 December 2022
                Categories
                Case Report
                Pancreas
                Custom metadata
                TRUE

                pancreatic neuroendocrine tumor,insulinoma,hepatic artery embolization,diazoxide,liver metastases

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