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Pancreatic endocrine tumor

MedGen UID:
66883
Concept ID:
C0242363
Neoplastic Process
Synonyms: Island Cell Tumor; Island Cell Tumors; Islet Cell Tumor; Islet Cell Tumors; Islet of Langerhans Tumor; Langerhans Tumor Islet; Nesidioblastoma; Nesidioblastomas; Pancreatic Islet Cell Tumors; Tumor Islet, Langerhans; Tumor, Island Cell; Tumor, Islet Cell; Tumors, Island Cell; Tumors, Islet Cell
SNOMED CT: Nesidioblastoma (76345009); Neoplasm of islets of Langerhans (126864006); Islet cell neoplasm (399528006); Islet cell tumor (128878003); Pancreatic endocrine tumor (128878003)
 
HPO: HP:0030405

Definition

A neuroendocrine tumor originating in a hormone-producing cell (islet cell) of the pancreas. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic endocrine tumor

Conditions with this feature

Multiple endocrine neoplasia type 4
MedGen UID:
373469
Concept ID:
C1970712
Neoplastic Process
Multiple endocrine neoplasia is a group of disorders that affect the body's network of hormone-producing glands called the endocrine system. Hormones are chemical messengers that travel through the bloodstream and regulate the function of cells and tissues throughout the body. Multiple endocrine neoplasia typically involves tumors (neoplasia) in at least two endocrine glands; tumors can also develop in other organs and tissues. These growths can be noncancerous (benign) or cancerous (malignant). If the tumors become cancerous, the condition can be life-threatening.\n\nThe major forms of multiple endocrine neoplasia are called type 1, type 2, and type 4. These types are distinguished by the genes involved, the types of hormones made, and the characteristic signs and symptoms.\n\nMany different types of tumors are associated with multiple endocrine neoplasia. Type 1 frequently involves tumors of the parathyroid glands, the pituitary gland, and the pancreas. Tumors in these glands can lead to the overproduction of hormones. The most common sign of multiple endocrine neoplasia type 1 is overactivity of the parathyroid glands (hyperparathyroidism). Hyperparathyroidism disrupts the normal balance of calcium in the blood, which can lead to kidney stones, thinning of bones, nausea and vomiting, high blood pressure (hypertension), weakness, and fatigue.\n\nMultiple endocrine neoplasia type 4 appears to have signs and symptoms similar to those of type 1, although it is caused by mutations in a different gene. Hyperparathyroidism is the most common feature, followed by tumors of the pituitary gland, additional endocrine glands, and other organs.\n\nThe most common sign of multiple endocrine neoplasia type 2 is a form of thyroid cancer called medullary thyroid carcinoma. Some people with this disorder also develop a pheochromocytoma, which is an adrenal gland tumor that can cause dangerously high blood pressure. Multiple endocrine neoplasia type 2 is divided into three subtypes: type 2A, type 2B (formerly called type 3), and familial medullary thyroid carcinoma (FMTC). These subtypes differ in their characteristic signs and symptoms and risk of specific tumors; for example, hyperparathyroidism occurs only in type 2A, and medullary thyroid carcinoma is the only feature of FMTC. The signs and symptoms of multiple endocrine neoplasia type 2 are relatively consistent within any one family.

Professional guidelines

PubMed

Ito T, Lee L, Jensen RT
Expert Opin Pharmacother 2016 Nov;17(16):2191-2205. Epub 2016 Sep 23 doi: 10.1080/14656566.2016.1236916. PMID: 27635672Free PMC Article
Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P, Scoazec JY, Salazar R, Sauvanet A, Kianmanesh R; Barcelona Consensus Conference participants
Neuroendocrinology 2012;95(2):98-119. Epub 2012 Feb 15 doi: 10.1159/000335591. PMID: 22261919Free PMC Article
Jürgensen C, Neser F, Stölzel U
Endoscopy 2008 Nov;40(11):963; author reply 963. Epub 2008 Nov 13 doi: 10.1055/s-2008-1077632. PMID: 19009494

Recent clinical studies

Etiology

Kos-Kudła B, Castaño JP, Denecke T, Grande E, Kjaer A, Koumarianou A, de Mestier L, Partelli S, Perren A, Stättner S, Valle JW, Fazio N
J Neuroendocrinol 2023 Dec;35(12):e13343. Epub 2023 Oct 25 doi: 10.1111/jne.13343. PMID: 37877341
Dąbkowski K, Starzyńska T
Endokrynol Pol 2023;74(1):25-30. Epub 2022 Dec 15 doi: 10.5603/EP.a2022.0080. PMID: 36519652
Evans LM, Geenen KR, O'Shea A, Hedgire SS, Ferrone CR, Thiele EA
Am J Med Genet A 2022 Sep;188(9):2666-2671. Epub 2022 May 25 doi: 10.1002/ajmg.a.62850. PMID: 35612824
Beane JD, Borrebach JD, Billderback A, Onuma AE, Adam MA, Zureikat AH, Pitt HA
Am J Surg 2021 Jul;222(1):29-34. Epub 2020 Dec 8 doi: 10.1016/j.amjsurg.2020.12.013. PMID: 33317810
Kuo JH, Lee JA, Chabot JA
Surg Clin North Am 2014 Jun;94(3):689-708. Epub 2014 Apr 13 doi: 10.1016/j.suc.2014.02.010. PMID: 24857584

Diagnosis

Acher AW, Hallet J
Surg Clin North Am 2024 Oct;104(5):1095-1111. Epub 2024 Apr 23 doi: 10.1016/j.suc.2024.03.010. PMID: 39237166
Kos-Kudła B, Castaño JP, Denecke T, Grande E, Kjaer A, Koumarianou A, de Mestier L, Partelli S, Perren A, Stättner S, Valle JW, Fazio N
J Neuroendocrinol 2023 Dec;35(12):e13343. Epub 2023 Oct 25 doi: 10.1111/jne.13343. PMID: 37877341
Ito T, Lee L, Jensen RT
Expert Opin Pharmacother 2016 Nov;17(16):2191-2205. Epub 2016 Sep 23 doi: 10.1080/14656566.2016.1236916. PMID: 27635672Free PMC Article
Kuo JH, Lee JA, Chabot JA
Surg Clin North Am 2014 Jun;94(3):689-708. Epub 2014 Apr 13 doi: 10.1016/j.suc.2014.02.010. PMID: 24857584
Jensen RT, Cadiot G, Brandi ML, de Herder WW, Kaltsas G, Komminoth P, Scoazec JY, Salazar R, Sauvanet A, Kianmanesh R; Barcelona Consensus Conference participants
Neuroendocrinology 2012;95(2):98-119. Epub 2012 Feb 15 doi: 10.1159/000335591. PMID: 22261919Free PMC Article

Therapy

Beane JD, Borrebach JD, Billderback A, Onuma AE, Adam MA, Zureikat AH, Pitt HA
Am J Surg 2021 Jul;222(1):29-34. Epub 2020 Dec 8 doi: 10.1016/j.amjsurg.2020.12.013. PMID: 33317810
Ito T, Lee L, Jensen RT
Expert Opin Pharmacother 2016 Nov;17(16):2191-2205. Epub 2016 Sep 23 doi: 10.1080/14656566.2016.1236916. PMID: 27635672Free PMC Article
Muscatiello N, Salcuni A, Macarini L, Cignarelli M, Prencipe S, di Maso M, Castriota M, D'Agnessa V, Ierardi E
Endoscopy 2008 Sep;40 Suppl 2:E258-9. Epub 2008 Dec 17 doi: 10.1055/s-2007-966962. PMID: 19090457
Muscatiello N, Nacchiero M, Della Valle N, Di Terlizzi F, Verderosa G, Salcuni A, Macarini L, Cignarelli M, Castriota M, D'Agnessa V, Ierardi E
Endoscopy 2008 Sep;40 Suppl 2:E83. Epub 2008 Mar 20 doi: 10.1055/s-2007-995540. PMID: 18633893
Hamoud AK, Khan MF, Aboalmaali N, Usadel KH, Wullstein C, Vogl TJ
Eur Radiol 2004 May;14(5):923-5. Epub 2003 Sep 3 doi: 10.1007/s00330-003-2017-2. PMID: 12955450

Prognosis

Andreasi V, Partelli S, Schiavo Lena M, Muffatti F, Battistella A, Tamburrino D, Pecorelli N, Crippa S, Balzano G, Doglioni C, Falconi M
Br J Surg 2024 Apr 3;111(4) doi: 10.1093/bjs/znae076. PMID: 38682424Free PMC Article
Kuo JH, Lee JA, Chabot JA
Surg Clin North Am 2014 Jun;94(3):689-708. Epub 2014 Apr 13 doi: 10.1016/j.suc.2014.02.010. PMID: 24857584
Rustagi T, Rai M, Bauer F
J Gastrointest Cancer 2013 Sep;44(3):336-42. doi: 10.1007/s12029-012-9445-7. PMID: 23065708
Jensen RT, Berna MJ, Bingham DB, Norton JA
Cancer 2008 Oct 1;113(7 Suppl):1807-43. doi: 10.1002/cncr.23648. PMID: 18798544Free PMC Article
Gibril F, Jensen RT
Curr Gastroenterol Rep 2005 May;7(2):114-21. doi: 10.1007/s11894-005-0049-2. PMID: 15802099

Clinical prediction guides

Hijioka S, Yamashige D, Esaki M, Honda G, Higuchi R, Masui T, Shimizu Y, Ohtsuka M, Kumamoto Y, Katanuma A, Gotohda N, Akita H, Unno M, Endo I, Yokoyama Y, Yamada S, Matsumoto I, Ohtsuka T, Hirano S, Yasuda H, Kawai M, Aoki T, Nakamura M, Hashimoto D, Rikiyama T, Horiguchi A, Fujii T, Mizuno S, Hanada K, Tani M, Hatori T, Ito T, Okuno M, Kagawa S, Tajima H, Ishii T, Sugimoto M, Onoe S, Takami H, Takada R, Miura T, Kurita Y, Kamei K, Mataki Y, Okazaki K, Takeyama Y, Yamaue H, Satoi S; Japan Pancreas Society Clinical Research Promotion Committee Group (Corporate Authorship)
Clin Gastroenterol Hepatol 2024 Jul;22(7):1416-1426.e5. Epub 2024 Apr 13 doi: 10.1016/j.cgh.2024.03.029. PMID: 38615727
Okawa Y, Tsuchikawa T, Hatanaka KC, Matsui A, Tanaka K, Nakanishi Y, Asano T, Noji T, Nakamura T, Mitsuhashi T, Okamura K, Hatanaka Y, Hirano S
Pancreas 2022 Apr 1;51(4):338-344. Epub 2022 Jun 14 doi: 10.1097/MPA.0000000000002029. PMID: 35699685
Marx M, Godat S, Caillol F, Poizat F, Ratone JP, Pesenti C, Schoepfer A, Hoibian S, Dahel Y, Giovannini M
Dig Endosc 2022 Sep;34(6):1207-1213. Epub 2022 Jan 19 doi: 10.1111/den.14224. PMID: 34963025
Ricci C, Partelli S, Landoni L, Rinzivillo M, Ingaldi C, Andreasi V, Nessi C, Muffatti F, Fontana M, Tamburrino D, Deiro G, Alberici L, Campana D, Panzuto F, Bassi C, Falconi M, Casadei R
Br J Surg 2021 Jul 23;108(7):811-816. doi: 10.1093/bjs/znaa141. PMID: 33724300
Deguelte S, de Mestier L, Hentic O, Cros J, Lebtahi R, Hammel P, Kianmanesh R
J Visc Surg 2018 Dec;155(6):483-492. Epub 2018 Nov 15 doi: 10.1016/j.jviscsurg.2018.08.010. PMID: 30448206

Recent systematic reviews

Wei K, Zheng Q, Cheng L, Li W, Nießen A, Uzunoglu FG, Nickel F, Tian J, Wang Z, Hackert T
Int J Surg 2024 Dec 1;110(12):8250-8255. doi: 10.1097/JS9.0000000000002143. PMID: 39806757Free PMC Article
Clarke CN, Ward E, Henry V, Nimmer K, Phan A, Evans DB
Ann Surg Oncol 2024 Aug;31(8):4976-4985. Epub 2024 Apr 23 doi: 10.1245/s10434-024-15249-1. PMID: 38652199
Guo J, Zhao J, Bi X, Li Z, Huang Z, Zhang Y, Cai J, Zhao H
Oncotarget 2017 May 23;8(21):35368-35375. doi: 10.18632/oncotarget.15685. PMID: 28430639Free PMC Article

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