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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
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

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 Cardiovasc Imaging 2022 Sep 10;23(10):e333-e465. doi: 10.1093/ehjci/jeac106. PMID: 36017575
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
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
Srirajaskanthan R, Shanmugabavan D, Ramage JK
BMJ 2010 Aug 23;341:c3941. doi: 10.1136/bmj.c3941. PMID: 20732967
Tilson MD
Surg Clin North Am 1974 Apr;54(2):409-23. doi: 10.1016/s0039-6109(16)40288-4. PMID: 4593912

Therapy

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 Cardiovasc Imaging 2022 Sep 10;23(10):e333-e465. doi: 10.1093/ehjci/jeac106. PMID: 36017575
Fernandez-Robles C, Carr ZJ, Oprea AD
Curr Opin Anaesthesiol 2021 Jun 1;34(3):326-334. doi: 10.1097/ACO.0000000000000986. PMID: 33935181
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
Tilson MD
Surg Clin North Am 1974 Apr;54(2):409-23. doi: 10.1016/s0039-6109(16)40288-4. PMID: 4593912

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
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
Kulke MH, Mayer RJ
N Engl J Med 1999 Mar 18;340(11):858-68. doi: 10.1056/NEJM199903183401107. PMID: 10080850
Tilson MD
Surg Clin North Am 1974 Apr;54(2):409-23. doi: 10.1016/s0039-6109(16)40288-4. PMID: 4593912

Clinical prediction guides

Chauhan A, Das S, Miller R, Luque L, Cheuvront SN, Cloud J, Tarter Z, Siddiqui F, Ramirez RA, Anthony L
BMC Cancer 2021 May 20;21(1):580. doi: 10.1186/s12885-021-08315-4. PMID: 34016080Free PMC Article
Joish VN, Shah S, Tierce JC, Patel D, McKee C, Lapuerta P, Zacks J
Future Oncol 2019 Apr;15(12):1397-1406. Epub 2019 Feb 8 doi: 10.2217/fon-2018-0960. PMID: 30734573
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
Paragliola RM, Prete A, Papi G, Torino F, Corsello A, Pontecorvi A, Corsello SM
Drug Des Devel Ther 2016;10:3459-3470. Epub 2016 Oct 25 doi: 10.2147/DDDT.S76732. PMID: 27822010Free PMC Article
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

Recent systematic reviews

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
Joish VN, Shah S, Tierce JC, Patel D, McKee C, Lapuerta P, Zacks J
Future Oncol 2019 Apr;15(12):1397-1406. Epub 2019 Feb 8 doi: 10.2217/fon-2018-0960. PMID: 30734573
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
Pusceddu S, De Braud F, Festinese F, Bregant C, Lorenzoni A, Maccauro M, Milione M, Concas L, Formisano B, Leuzzi L, Mazzaferro V, Buzzoni R
Future Oncol 2015;11(13):1947-59. doi: 10.2217/fon.15.86. PMID: 26161929
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

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