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Carcinoid syndrome

MedGen UID:
6191
Concept ID:
C0024586
Disease or Syndrome
Synonym: Carcinoid tumor syndrome
SNOMED CT: Hormonal tumor (35868009); Excessive serotonin secretion (35868009); Carcinoid syndrome (35868009); Hormone secretion by carcinoid tumor (35868009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0100347
Orphanet: ORPHA100093

Definition

A rare neoplastic disease characterized by the occurrence of a hormonal syndrome resulting from secretion of humoral factors (including polypeptides, vasoactive amines, and prostaglandins) from a functional neuroendocrine tumor (particularly from the midgut), typically manifesting with increased bowel movements and diarrhea, episodic vasoactive flushes (particularly of the face), hypotension, tachycardia, venous telangiectasia, dyspnea, and bronchospasms, as well as long-term fibrotic changes in the mesentery, retroperitoneum, and of the cardiac valves. [from ORDO]

Term Hierarchy

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

Professional guidelines

PubMed

Lyon AR, López-Fernández T, Couch LS, Asteggiano R, Aznar MC, Bergler-Klein J, Boriani G, Cardinale D, Cordoba R, Cosyns B, Cutter DJ, de Azambuja E, de Boer RA, Dent SF, Farmakis D, Gevaert SA, Gorog DA, Herrmann J, Lenihan D, Moslehi J, Moura B, Salinger SS, Stephens R, Suter TM, Szmit S, Tamargo J, Thavendiranathan P, Tocchetti CG, van der Meer P, van der Pal HJH; ESC Scientific Document Group
Eur Heart J 2022 Nov 1;43(41):4229-4361. doi: 10.1093/eurheartj/ehac244. PMID: 36017568
Jin C, Sharma AN, Thevakumar B, Majid M, Al Chalaby S, Takahashi N, Tanious A, Arockiam AD, Beri N, Amsterdam EA
Cardiology 2021;146(1):65-73. Epub 2020 Oct 16 doi: 10.1159/000507847. PMID: 33070143
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366

Recent clinical studies

Etiology

Lyon AR, López-Fernández T, Couch LS, Asteggiano R, Aznar MC, Bergler-Klein J, Boriani G, Cardinale D, Cordoba R, Cosyns B, Cutter DJ, de Azambuja E, de Boer RA, Dent SF, Farmakis D, Gevaert SA, Gorog DA, Herrmann J, Lenihan D, Moslehi J, Moura B, Salinger SS, Stephens R, Suter TM, Szmit S, Tamargo J, Thavendiranathan P, Tocchetti CG, van der Meer P, van der Pal HJH; ESC Scientific Document Group
Eur Heart J 2022 Nov 1;43(41):4229-4361. doi: 10.1093/eurheartj/ehac244. PMID: 36017568
LoVerde D, Iweala OI, Eginli A, Krishnaswamy G
Chest 2018 Feb;153(2):528-543. Epub 2017 Aug 8 doi: 10.1016/j.chest.2017.07.033. PMID: 28800865Free PMC Article
Öberg KE
Ann Oncol 2010 Oct;21 Suppl 7:vii72-80. doi: 10.1093/annonc/mdq290. PMID: 20943646
Pasieka JL
Surg Clin North Am 2009 Oct;89(5):1123-37. doi: 10.1016/j.suc.2009.06.008. PMID: 19836488
Foti R, Leonardi R, Rondinone R, Di Gangi M, Leonetti C, Canova M, Doria A
Autoimmun Rev 2008 Feb;7(4):331-9. Epub 2008 Jan 11 doi: 10.1016/j.autrev.2007.12.004. PMID: 18295739

Diagnosis

Fernandes CJ, Leung G, Eads JR, Katona BW
Gastroenterol Clin North Am 2022 Sep;51(3):625-647. Epub 2022 Aug 30 doi: 10.1016/j.gtc.2022.06.002. PMID: 36153114
Peramunage D, Nikravan S
Anesthesiol Clin 2020 Mar;38(1):149-163. Epub 2020 Jan 2 doi: 10.1016/j.anclin.2019.10.006. PMID: 32008649
Ito T, Lee L, Jensen RT
Curr Opin Endocrinol Diabetes Obes 2018 Feb;25(1):22-35. doi: 10.1097/MED.0000000000000376. PMID: 29120923Free PMC Article
Hassan SA, Banchs J, Iliescu C, Dasari A, Lopez-Mattei J, Yusuf SW
Heart 2017 Oct;103(19):1488-1495. Epub 2017 Jun 8 doi: 10.1136/heartjnl-2017-311261. PMID: 28596302
Bendelow J, Apps E, Jones LE, Poston GJ
Eur J Surg Oncol 2008 Mar;34(3):289-96. Epub 2007 Dec 18 doi: 10.1016/j.ejso.2007.07.202. PMID: 18068329

Therapy

Kalra S, Bhattacharya S, Dhingra A, Khare J, Jindal S
J Pak Med Assoc 2024 May;74(5):998-999. doi: 10.47391/JPMA.24-36. PMID: 38783456
Cives M, Strosberg JR
CA Cancer J Clin 2018 Nov;68(6):471-487. Epub 2018 Oct 8 doi: 10.3322/caac.21493. PMID: 30295930
Ito T, Lee L, Jensen RT
Curr Opin Endocrinol Diabetes Obes 2018 Feb;25(1):22-35. doi: 10.1097/MED.0000000000000376. PMID: 29120923Free PMC Article
Basuroy R, Srirajaskanthan R, Ramage JK
Gastroenterol Clin North Am 2016 Sep;45(3):487-507. doi: 10.1016/j.gtc.2016.04.007. PMID: 27546845
Bendelow J, Apps E, Jones LE, Poston GJ
Eur J Surg Oncol 2008 Mar;34(3):289-96. Epub 2007 Dec 18 doi: 10.1016/j.ejso.2007.07.202. PMID: 18068329

Prognosis

Cives M, Strosberg JR
CA Cancer J Clin 2018 Nov;68(6):471-487. Epub 2018 Oct 8 doi: 10.3322/caac.21493. PMID: 30295930
Ito T, Lee L, Jensen RT
Curr Opin Endocrinol Diabetes Obes 2018 Feb;25(1):22-35. doi: 10.1097/MED.0000000000000376. PMID: 29120923Free PMC Article
Hassan SA, Banchs J, Iliescu C, Dasari A, Lopez-Mattei J, Yusuf SW
Heart 2017 Oct;103(19):1488-1495. Epub 2017 Jun 8 doi: 10.1136/heartjnl-2017-311261. PMID: 28596302
Basuroy R, Srirajaskanthan R, Ramage JK
Gastroenterol Clin North Am 2016 Sep;45(3):487-507. doi: 10.1016/j.gtc.2016.04.007. PMID: 27546845
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366

Clinical prediction guides

Pan IW, Halperin DM, Kim B, Yao JC, Shih YT
Pharmacoeconomics 2021 Nov;39(11):1271-1297. Epub 2021 Aug 11 doi: 10.1007/s40273-021-01071-0. PMID: 34378163Free PMC Article
Cives M, Strosberg JR
CA Cancer J Clin 2018 Nov;68(6):471-487. Epub 2018 Oct 8 doi: 10.3322/caac.21493. PMID: 30295930
Bernheim AM, Connolly HM, Hobday TJ, Abel MD, Pellikka PA
Prog Cardiovasc Dis 2007 May-Jun;49(6):439-51. doi: 10.1016/j.pcad.2006.12.002. PMID: 17498524
Schnirer II, Yao JC, Ajani JA
Acta Oncol 2003;42(7):672-92. doi: 10.1080/02841860310010547. PMID: 14690153
Moertel CG
J Clin Oncol 1983 Nov;1(11):727-40. doi: 10.1200/JCO.1983.1.11.727. PMID: 6199469

Recent systematic reviews

Mulders MCF, de Herder WW, Hofland J
Endocr Rev 2024 May 7;45(3):351-360. doi: 10.1210/endrev/bnad035. PMID: 38038364Free PMC Article
Pan IW, Halperin DM, Kim B, Yao JC, Shih YT
Pharmacoeconomics 2021 Nov;39(11):1271-1297. Epub 2021 Aug 11 doi: 10.1007/s40273-021-01071-0. PMID: 34378163Free PMC Article
Wolin EM, Benson Iii AB
Oncology 2019;96(6):273-289. Epub 2019 Apr 24 doi: 10.1159/000499049. PMID: 31018209
Hofland J, Herrera-Martínez AD, Zandee WT, de Herder WW
Endocr Relat Cancer 2019 Mar;26(3):R145-R156. doi: 10.1530/ERC-18-0495. PMID: 30608900
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366

Supplemental Content

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