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Carcinoid tumor

MedGen UID:
2838
Concept ID:
C0007095
Neoplastic Process
Synonym: Carcinoid tumor (disease)
SNOMED CT: Carcinoid tumor (443492008); Carcinoid tumor (189607006); Carcinoid (189607006); Carcinoid tumor - morphology (189607006)
 
HPO: HP:0100570
Monarch Initiative: MONDO:0005369

Definition

A tumor formed from the endocrine (argentaffin) cells of the mucosal lining of a variety of organs including the stomach and intestine. These cells are from neuroectodermal origin. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCarcinoid tumor

Conditions with this feature

Multiple endocrine neoplasia, type 1
MedGen UID:
9957
Concept ID:
C0025267
Neoplastic Process
Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of more than 20 endocrine and non-endocrine tumors. Endocrine tumors become evident either by overproduction of hormones by the tumor or by growth of the tumor itself. Parathyroid tumors are the main MEN1-associated endocrinopathy; onset in 90% of individuals is between ages 20 and 25 years with hypercalcemia evident by age 50 years; hypercalcemia causes lethargy, depression, confusion, anorexia, constipation, nausea, vomiting, diuresis, dehydration, hypercalciuria, kidney stones, increased bone resorption/fracture risk, hypertension, and shortened QT interval. Pituitary tumors include prolactinoma (the most common), which manifests as oligomenorrhea/amenorrhea and galactorrhea in females and sexual dysfunction in males. Well-differentiated endocrine tumors of the gastro-entero-pancreatic (GEP) tract can manifest as Zollinger-Ellison syndrome (gastrinoma); hypoglycemia (insulinoma); hyperglycemia, anorexia, glossitis, anemia, diarrhea, venous thrombosis, and skin rash (glucagonoma); and watery diarrhea, hypokalemia, and achlorhydria syndrome (vasoactive intestinal peptide [VIP]-secreting tumor). Carcinoid tumors are non-hormone-secreting and can manifest as a large mass after age 50 years. Adrenocortical tumors can be associated with primary hypercortisolism or hyperaldosteronism. Non-endocrine tumors include facial angiofibromas, collagenomas, lipomas, meningiomas, ependymomas, and leiomyomas.
Neurofibromatosis-pheochromocytoma-duodenal carcinoid syndrome
MedGen UID:
331696
Concept ID:
C1834232
Disease or Syndrome
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.

Recent clinical studies

Etiology

Villaescusa M, Andres MP, Amaral AC, Barbosa RN, Abrão MS
Minerva Obstet Gynecol 2021 Oct;73(5):606-613. Epub 2021 Apr 27 doi: 10.23736/S2724-606X.21.04792-4. PMID: 33904692
Widmeier E, Füllgraf H, Waller CF
BMC Urol 2020 Dec 14;20(1):197. doi: 10.1186/s12894-020-00768-2. PMID: 33317491Free PMC Article
Gupta A, Lubner MG, Wertz RM, Foley E, Loeffler A, Pickhardt PJ
Abdom Radiol (NY) 2019 Aug;44(8):2721-2728. doi: 10.1007/s00261-019-01945-0. PMID: 31016344
Herde RF, Kokeny KE, Reddy CB, Akerley WL, Hu N, Boltax JP, Hitchcock YJ
Am J Clin Oncol 2018 Jan;41(1):24-29. doi: 10.1097/COC.0000000000000221. PMID: 26270444Free PMC Article
Choi CW, Park SB, Kang DH, Kim HW, Kim SJ, Nam HS, Ryu DG
Surg Endosc 2017 Dec;31(12):5006-5011. Epub 2017 Sep 21 doi: 10.1007/s00464-017-5497-x. PMID: 28936630

Diagnosis

Widmeier E, Füllgraf H, Waller CF
BMC Urol 2020 Dec 14;20(1):197. doi: 10.1186/s12894-020-00768-2. PMID: 33317491Free PMC Article
Song IH, Hong SH, Lee KY, Kang J, Lee SH, Lee J, Lee A
Pathol Res Pract 2020 May;216(5):152835. Epub 2020 Jan 20 doi: 10.1016/j.prp.2020.152835. PMID: 31983568
Hsu WW, Mao TL, Chen CH
Taiwan J Obstet Gynecol 2019 Jul;58(4):570-573. doi: 10.1016/j.tjog.2019.05.025. PMID: 31307754
Darré T, Doukouré B, Kouyaté M, Djiwa T, Kwamé D, Napo-Koura G
Tumori 2019 Dec;105(6):NP20-NP23. Epub 2019 Feb 24 doi: 10.1177/0300891619832263. PMID: 30799770
Koustais S, O'Halloran PJ, Hassan A, Brett F, Young S
World Neurosurg 2017 Sep;105:1042.e11-1042.e14. Epub 2017 Jul 11 doi: 10.1016/j.wneu.2017.06.179. PMID: 28705700

Therapy

Kaplun O, Papamanoli A, Chernyavskiy I, Psevdos G
Indian J Pathol Microbiol 2021 Apr-Jun;64(2):413-414. doi: 10.4103/IJPM.IJPM_187_20. PMID: 33851649
Bayat Mokhtari R, Baluch N, Morgatskaya E, Kumar S, Sparaneo A, Muscarella LA, Zhao S, Cheng HL, Das B, Yeger H
BMC Cancer 2019 Aug 30;19(1):864. doi: 10.1186/s12885-019-6018-1. PMID: 31470802Free PMC Article
Shin TH, Inagaki E, Ganta T, Hartshorn K, Litle VR, Suzuki K
Ann Thorac Surg 2019 Mar;107(3):e199-e201. Epub 2018 Sep 12 doi: 10.1016/j.athoracsur.2018.06.089. PMID: 30218665
Choi CW, Park SB, Kang DH, Kim HW, Kim SJ, Nam HS, Ryu DG
Surg Endosc 2017 Dec;31(12):5006-5011. Epub 2017 Sep 21 doi: 10.1007/s00464-017-5497-x. PMID: 28936630
Pleşa A, Sarca E, Maxim R
Rev Med Chir Soc Med Nat Iasi 2014 Oct-Dec;118(4):1018-23. PMID: 25581963

Prognosis

Widmeier E, Füllgraf H, Waller CF
BMC Urol 2020 Dec 14;20(1):197. doi: 10.1186/s12894-020-00768-2. PMID: 33317491Free PMC Article
Hsu WW, Mao TL, Chen CH
Taiwan J Obstet Gynecol 2019 Jul;58(4):570-573. doi: 10.1016/j.tjog.2019.05.025. PMID: 31307754
Wang Z, Yang MQ, Huang WJ, Zhang D, Xu HT
Medicine (Baltimore) 2019 Feb;98(5):e14315. doi: 10.1097/MD.0000000000014315. PMID: 30702609Free PMC Article
Herde RF, Kokeny KE, Reddy CB, Akerley WL, Hu N, Boltax JP, Hitchcock YJ
Am J Clin Oncol 2018 Jan;41(1):24-29. doi: 10.1097/COC.0000000000000221. PMID: 26270444Free PMC Article
Choi CW, Park SB, Kang DH, Kim HW, Kim SJ, Nam HS, Ryu DG
Surg Endosc 2017 Dec;31(12):5006-5011. Epub 2017 Sep 21 doi: 10.1007/s00464-017-5497-x. PMID: 28936630

Clinical prediction guides

Chen X, Pang Z, Wang Y, Bie F, Zeng Y, Wang G, Du J
Lung Cancer 2020 Jan;139:94-102. Epub 2019 Nov 14 doi: 10.1016/j.lungcan.2019.11.006. PMID: 31759223
Huang Y, Yang X, Lu T, Li M, Zhao M, Yang X, Ma K, Wang S, Zhan C, Liu Y, Wang Q
Cancer Med 2018 Jun;7(6):2434-2441. Epub 2018 May 7 doi: 10.1002/cam4.1515. PMID: 29733505Free PMC Article
Seker KG, Sam E, Sahin S, Yenice MG, Aktas AG, Simsek A, Tugcu V
Arch Ital Urol Androl 2017 Dec 31;89(4):316-318. doi: 10.4081/aiua.2017.4.316. PMID: 29473385
Audlin JR, Khullar G, Deshaies EM, Kurra S, Lavelle WF
World Neurosurg 2016 Dec;96:607.e1-607.e6. Epub 2016 Oct 3 doi: 10.1016/j.wneu.2016.09.088. PMID: 27713062
Kim SS, Choi C, Kang TW, Choi YD
Pathol Int 2016 Jan;66(1):42-6. Epub 2015 Dec 8 doi: 10.1111/pin.12367. PMID: 26644387

Recent systematic reviews

Villaescusa M, Andres MP, Amaral AC, Barbosa RN, Abrão MS
Minerva Obstet Gynecol 2021 Oct;73(5):606-613. Epub 2021 Apr 27 doi: 10.23736/S2724-606X.21.04792-4. PMID: 33904692
Sluiter NR, van der Bilt JD, Croll DMR, Vriens MR, de Hingh IHJT, Hemmer P, Aalbers AGJ, Bremers AJA, Ceelen W, D'Hoore A, Schoonmade LJ, Coupé V, Verheul H, Kazemier G, Tuynman JB; Dutch Peritoneal Oncology Group.
Clin Colorectal Cancer 2020 Sep;19(3):e87-e99. Epub 2020 Jan 30 doi: 10.1016/j.clcc.2020.01.002. PMID: 32651131
Jiang Y, Hou G, Cheng W
Medicine (Baltimore) 2019 Mar;98(10):e14769. doi: 10.1097/MD.0000000000014769. PMID: 30855482Free PMC Article
Marchevsky AM, Hendifar A, Walts AE
Mod Pathol 2018 Oct;31(10):1523-1531. Epub 2018 May 25 doi: 10.1038/s41379-018-0076-9. PMID: 29802361
Wirtschafter E, Walts AE, Liu ST, Marchevsky AM
Lung 2015 Oct;193(5):659-67. Epub 2015 Jun 24 doi: 10.1007/s00408-015-9755-1. PMID: 26104490

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