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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]

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\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.\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\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.

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
Pavlidis TE, Psarras K, Symeonidis NG, Pavlidis ET, Sakantamis AK
Hepatobiliary Pancreat Dis Int 2011 Jun;10(3):243-7. doi: 10.1016/s1499-3872(11)60040-4. PMID: 21669565
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

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
Mei W, Cao F, Lu J, Qu C, Fang Z, Li J, Li F
Front Endocrinol (Lausanne) 2023;14:1140873. Epub 2023 Mar 20 doi: 10.3389/fendo.2023.1140873. PMID: 37020595Free 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
Antonello D, Gobbo S, Corbo V, Sipos B, Lemoine NR, Scarpa A
Pancreatology 2009;9(1-2):25-33. Epub 2008 Dec 12 doi: 10.1159/000178872. PMID: 19077452
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

Diagnosis

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
Davila A, Menias CO, Alhalabi K, Lall C, Pickhardt PJ, Lubner M, Elsayes KM
AJR Am J Roentgenol 2020 Oct;215(4):885-895. Epub 2020 Jul 13 doi: 10.2214/AJR.19.22542. PMID: 32755185
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
Dumlu EG, Karakoç D, Özdemir A
Int Surg 2015 Jun;100(6):1089-97. Epub 2015 Jan 15 doi: 10.9738/INTSURG-D-14-00204.1. PMID: 25590518Free 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

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
Wang BP, Tian WJ, Zhang J, Jiang CX, Qu HQ, Zhu M
J Int Med Res 2018 Feb;46(2):908-915. Epub 2017 Sep 14 doi: 10.1177/0300060517728653. PMID: 29239255Free PMC Article
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
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
van Vliet EI, van Eijck CH, de Krijger RR, Nieveen van Dijkum EJ, Teunissen JJ, Kam BL, de Herder WW, Feelders RA, Bonsing BA, Brabander T, Krenning EP, Kwekkeboom DJ
J Nucl Med 2015 Nov;56(11):1647-53. Epub 2015 Aug 13 doi: 10.2967/jnumed.115.158899. PMID: 26272813

Prognosis

Mei W, Cao F, Lu J, Qu C, Fang Z, Li J, Li F
Front Endocrinol (Lausanne) 2023;14:1140873. Epub 2023 Mar 20 doi: 10.3389/fendo.2023.1140873. PMID: 37020595Free PMC Article
van Beek DJ, Verschuur AVD, Brosens LAA, Valk GD, Pieterman CRC, Vriens MR
Surg Oncol Clin N Am 2023 Apr;32(2):343-371. doi: 10.1016/j.soc.2022.10.010. PMID: 36925190
Dumlu EG, Karakoç D, Özdemir A
Int Surg 2015 Jun;100(6):1089-97. Epub 2015 Jan 15 doi: 10.9738/INTSURG-D-14-00204.1. PMID: 25590518Free 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, Berna MJ, Bingham DB, Norton JA
Cancer 2008 Oct 1;113(7 Suppl):1807-43. doi: 10.1002/cncr.23648. PMID: 18798544Free PMC Article

Clinical prediction guides

Dąbkowski K, Starzyńska T
Endokrynol Pol 2023;74(1):25-30. Epub 2022 Dec 15 doi: 10.5603/EP.a2022.0080. PMID: 36519652
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
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
Mauriello C, Napolitano S, Gambardella C, Candela G, De Vita F, Orditura M, Sciascia V, Tartaglia E, Lanza M, Santini L, Conzo G
Int J Surg 2015 Sep;21 Suppl 1:S10-4. Epub 2015 Jun 26 doi: 10.1016/j.ijsu.2015.04.089. PMID: 26118605
Bettini R, Partelli S, Boninsegna L, Capelli P, Crippa S, Pederzoli P, Scarpa A, Falconi M
Surgery 2011 Jul;150(1):75-82. doi: 10.1016/j.surg.2011.02.022. PMID: 21683859

Recent systematic reviews

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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