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Pancreatic Alpha Cell Adenoma

MedGen UID:
83549
•Concept ID:
C0279730
•
Neoplastic Process

Definition

A benign endocrine neoplasm arising from the alpha cells of the pancreas. It produces glucagon. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic Alpha Cell Adenoma

Professional guidelines

PubMed

Therapeutic strategies in the management of endocrine GEP tumours.
Arnold R
Eur J Clin Invest 1990 Oct;20 Suppl 1:S82-90. doi: 10.1111/j.1365-2362.1990.tb01782.x. PMID: 1702388
Medical treatment of neuroendocrine gut and pancreatic tumors.
Oberg K, Eriksson B
Acta Oncol 1989;28(3):425-31. doi: 10.3109/02841868909111217. PMID: 2472826
Assessment of treatment of intrahepatic malignancies using chemotherapy via an implantable pump.
Schwartz SI, Jones LS, McCune CS
Ann Surg 1985 May;201(5):560-7. doi: 10.1097/00000658-198505000-00004. PMID: 2581518Free PMC Article

Recent clinical studies

Etiology

Prognostic transcriptome classes of duodenopancreatic neuroendocrine tumors.
Diedisheim M, Dermine S, Jouinot A, Septier A, Gaujoux S, Dousset B, Cadiot G, Larger E, Bertherat J, Scharfmann R, Terris B, Coriat R, Assié G
Endocr Relat Cancer 2021 Jun 21;28(8):563-571. doi: 10.1530/ERC-21-0051. PMID: 34061769
Cytohistological diagnosis of pancreatic serous cystadenoma: a multimodal approach.
Steel M, Rao S, Ho J, Donnellan F, Yang HM, Schaeffer DF
J Clin Pathol 2019 Sep;72(9):615-621. Epub 2019 Jun 23 doi: 10.1136/jclinpath-2019-205872. PMID: 31235542
Histological changes in endocrine and exocrine pancreatic tissue from patients exposed to incretin-based therapies.
Ueberberg S, Jütte H, Uhl W, Schmidt W, Nauck M, Montanya E, Tannapfel A, Meier J
Diabetes Obes Metab 2016 Dec;18(12):1253-1262. Epub 2016 Sep 26 doi: 10.1111/dom.12766. PMID: 27545110
Fine-needle aspirations of pancreatic serous cystadenomas: improving diagnostic yield with cell blocks and α-inhibin immunohistochemistry.
Salomao M, Remotti H, Allendorf JD, Poneros JM, Sethi A, Gonda TA, Saqi A
Cancer Cytopathol 2014 Jan;122(1):33-9. Epub 2013 Aug 12 doi: 10.1002/cncy.21347. PMID: 23939868
Medical treatment of neuroendocrine gut and pancreatic tumors.
Oberg K, Eriksson B
Acta Oncol 1989;28(3):425-31. doi: 10.3109/02841868909111217. PMID: 2472826

Diagnosis

Cytohistological diagnosis of pancreatic serous cystadenoma: a multimodal approach.
Steel M, Rao S, Ho J, Donnellan F, Yang HM, Schaeffer DF
J Clin Pathol 2019 Sep;72(9):615-621. Epub 2019 Jun 23 doi: 10.1136/jclinpath-2019-205872. PMID: 31235542
Fine-needle aspirations of pancreatic serous cystadenomas: improving diagnostic yield with cell blocks and α-inhibin immunohistochemistry.
Salomao M, Remotti H, Allendorf JD, Poneros JM, Sethi A, Gonda TA, Saqi A
Cancer Cytopathol 2014 Jan;122(1):33-9. Epub 2013 Aug 12 doi: 10.1002/cncy.21347. PMID: 23939868
Interferon-alpha and interferon-gamma differentially affect pancreatic beta-cell phenotype and function.
Baldeón ME, Chun T, Gaskins HR
Am J Physiol 1998 Jul;275(1):C25-32. doi: 10.1152/ajpcell.1998.275.1.C25. PMID: 9688831
Glucagonoma syndrome.
Hashizume T, Kiryu H, Noda K, Kano T, Nakano R
J Am Acad Dermatol 1988 Aug;19(2 Pt 2):377-83. doi: 10.1016/s0190-9622(88)70182-6. PMID: 2842381
Glucagonoma syndrome.
Bloom SR, Polak JM
Am J Med 1987 May 29;82(5B):25-36. doi: 10.1016/0002-9343(87)90424-4. PMID: 2884877

Therapy

Histological changes in endocrine and exocrine pancreatic tissue from patients exposed to incretin-based therapies.
Ueberberg S, Jütte H, Uhl W, Schmidt W, Nauck M, Montanya E, Tannapfel A, Meier J
Diabetes Obes Metab 2016 Dec;18(12):1253-1262. Epub 2016 Sep 26 doi: 10.1111/dom.12766. PMID: 27545110
Marked expansion of exocrine and endocrine pancreas with incretin therapy in humans with increased exocrine pancreas dysplasia and the potential for glucagon-producing neuroendocrine tumors.
Butler AE, Campbell-Thompson M, Gurlo T, Dawson DW, Atkinson M, Butler PC
Diabetes 2013 Jul;62(7):2595-604. Epub 2013 Mar 22 doi: 10.2337/db12-1686. PMID: 23524641Free PMC Article
Medical treatment of neuroendocrine gut and pancreatic tumors.
Oberg K, Eriksson B
Acta Oncol 1989;28(3):425-31. doi: 10.3109/02841868909111217. PMID: 2472826
Glucagonoma syndrome.
Bloom SR, Polak JM
Am J Med 1987 May 29;82(5B):25-36. doi: 10.1016/0002-9343(87)90424-4. PMID: 2884877
Somatostatin and the endocrine pancreas.
Gerich JE
Curr Top Mol Endocrinol 1976;3:127-43. doi: 10.1007/978-1-4684-2598-7_9. PMID: 211005

Prognosis

BAG3 induces α-SMA expression in human fibroblasts and its over-expression correlates with poorer survival in fibrotic cancer patients.
De Marco M, Del Papa N, Reppucci F, Iorio V, Basile A, Falco A, Iaccarino R, Brongo S, De Caro F, Capunzo M, Turco MC, Rosati A, Marzullo L
J Cell Biochem 2022 Jan;123(1):91-101. Epub 2021 Nov 6 doi: 10.1002/jcb.30171. PMID: 34741483Free PMC Article
Prognostic transcriptome classes of duodenopancreatic neuroendocrine tumors.
Diedisheim M, Dermine S, Jouinot A, Septier A, Gaujoux S, Dousset B, Cadiot G, Larger E, Bertherat J, Scharfmann R, Terris B, Coriat R, Assié G
Endocr Relat Cancer 2021 Jun 21;28(8):563-571. doi: 10.1530/ERC-21-0051. PMID: 34061769
Cytohistological diagnosis of pancreatic serous cystadenoma: a multimodal approach.
Steel M, Rao S, Ho J, Donnellan F, Yang HM, Schaeffer DF
J Clin Pathol 2019 Sep;72(9):615-621. Epub 2019 Jun 23 doi: 10.1136/jclinpath-2019-205872. PMID: 31235542
Gastrointestinal hormones stimulate growth of Foregut Neuroendocrine Tumors by transactivating the EGF receptor.
Di Florio A, Sancho V, Moreno P, Delle Fave G, Jensen RT
Biochim Biophys Acta 2013 Mar;1833(3):573-82. Epub 2012 Dec 4 doi: 10.1016/j.bbamcr.2012.11.021. PMID: 23220008Free PMC Article
Adhesion receptors in malignant transformation and dissemination of gastrointestinal tumors.
Streit M, Schmidt R, Hilgenfeld RU, Thiel E, Kreuser ED
J Mol Med (Berl) 1996 May;74(5):253-68. doi: 10.1007/BF00196578. PMID: 8773262

Clinical prediction guides

Cytohistological diagnosis of pancreatic serous cystadenoma: a multimodal approach.
Steel M, Rao S, Ho J, Donnellan F, Yang HM, Schaeffer DF
J Clin Pathol 2019 Sep;72(9):615-621. Epub 2019 Jun 23 doi: 10.1136/jclinpath-2019-205872. PMID: 31235542
Clinical assessment of the GNAS mutation status in patients with intraductal papillary mucinous neoplasm of the pancreas.
Ohtsuka T, Tomosugi T, Kimura R, Nakamura S, Miyasaka Y, Nakata K, Mori Y, Morita M, Torata N, Shindo K, Ohuchida K, Nakamura M
Surg Today 2019 Nov;49(11):887-893. Epub 2019 Mar 16 doi: 10.1007/s00595-019-01797-7. PMID: 30879148
Interferon-alpha and interferon-gamma differentially affect pancreatic beta-cell phenotype and function.
Baldeón ME, Chun T, Gaskins HR
Am J Physiol 1998 Jul;275(1):C25-32. doi: 10.1152/ajpcell.1998.275.1.C25. PMID: 9688831
Adhesion receptors in malignant transformation and dissemination of gastrointestinal tumors.
Streit M, Schmidt R, Hilgenfeld RU, Thiel E, Kreuser ED
J Mol Med (Berl) 1996 May;74(5):253-68. doi: 10.1007/BF00196578. PMID: 8773262
Medical treatment of neuroendocrine gut and pancreatic tumors.
Oberg K, Eriksson B
Acta Oncol 1989;28(3):425-31. doi: 10.3109/02841868909111217. PMID: 2472826

Supplemental Content

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