Carcinoid syndrome

References

Key articles

National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

Singh S, Asa SL, Dey C, et al. Diagnosis and management of gastrointestinal neuroendocrine tumors: an evidence-based Canadian consensus. Cancer Treat Rev. 2016 Jun;47:32-45.Full text  Abstract

Riechelmann RP, Pereira AA, Rego JF, et al. Refractory carcinoid syndrome: a review of treatment options. Ther Adv Med Oncol. 2017 Feb;9(2):127-37.Full text  Abstract

Hofland J, Herrera-Martínez AD, Zandee WT, et al. Management of carcinoid syndrome: a systematic review and meta-analysis. Endocr Relat Cancer. 2019 Mar;26(3):R145-56.Full text  Abstract

Reference articles

1. Norheim I, Oberg K, Theodorsson-Norheim E, et al. Malignant carcinoid tumors. An analysis of 103 patients with regard to tumor localization, hormone production, and survival. Ann Surg. 1987 Aug;206(2):115-25.Full text  Abstract

2. Perez EA, Koniaris LG, Snell SE, et al. 7201 carcinoids: increasing incidence overall and disproportionate mortality in the elderly. World J Surg. 2007 May;31(5):1022-30. Abstract

3. Das S, Dasari A. Epidemiology, incidence, and prevalence of neuroendocrine neoplasms: are there global differences? Curr Oncol Rep. 2021 Mar 14;23(4):43.Full text  Abstract

4. Halperin DM, Shen C, Dasari A, et al. Frequency of carcinoid syndrome at neuroendocrine tumour diagnosis: a population-based study. Lancet Oncol. 2017 Apr;18(4):525-34.Full text  Abstract

5. Genus TSE, Bouvier C, Wong KF, et al. Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013-2015. Br J Cancer. 2019 Nov;121(11):966-72.Full text  Abstract

6. Rindi G, Bordi C. Endocrine tumours of the gastrointestinal tract: aetiology, molecular pathogenesis and genetics. Best Pract Res Clin Gastroenterol. 2005 Aug;19(4):519-34. Abstract

7. Mignon M. Natural history of neuroendocrine enteropancreatic tumors. Digestion. 2000;62(suppl 1):51-8. Abstract

8. Clement D, Ramage J, Srirajaskanthan R. Update on pathophysiology, treatment, and complications of carcinoid syndrome. J Oncol. 2020;2020:8341426.Full text  Abstract

9. Modlin IM, Oberg K, Chung DC, et al. Gastroenteropancreatic neuroendocrine tumours. Lancet Oncol. 2008 Jan;9(1):61-72. Abstract

10. de Herder WW. Biochemistry of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007 Mar;21(1):33-41. Abstract

11. Caplin ME, Buscombe JR, Hilson AJ, et al. Carcinoid tumour. Lancet. 1998 Sep 5;352(9130):799-805. Abstract

12. Phan AT, Oberg K, Choi J, et al; The North American Neuroendocrine Tumor Society (NANETS). NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the thorax (includes lung and thymus). Pancreas. 2010 Aug;39(6):784-98.Full text  Abstract

13. Buchmann I, Henze M, Engelbrecht S, et al. Comparison of 68Ga-DOTATOC PET and 111In-DTPAOC (Octreoscan) SPECT in patients with neuroendocrine tumours. Eur J Nucl Med Mol Imaging. 2007 Oct;34(10):1617-26. Abstract

14. Gabriel M, Decristoforo C, Kendler D, et al. 68Ga-DOTA-Tyr3-octreotide PET in neuroendocrine tumors: comparison with somatostatin receptor scintigraphy and CT. J Nucl Med. 2007 Apr;48(4):508-18.Full text  Abstract

15. Kaltsas G, Grossman A. Clinical features of gastroenteropancreatic tumours. In: Caplin ME, Kvols L, eds. Handbook of neuroendocrine tumours. Bristol, UK: BioScientifica; 2006:53-82.

16. Jameson JL, De Groot LJ, Giudice LC. Endocrinology: adult and pediatric. 7th ed. Philadelphia (Pa.). Saunders. 2016.Full text

17. Moinuddin M, Dean P, Vander Zwaag R, et al. The limitation of liver function tests in metastatic carcinoid tumors. Cancer. 1987 Apr 1;59(7):1304-6.Full text  Abstract

18. Bosman FT, Carneiro F, Hruban RH, et al. WHO Classification of tumours of the digestive system. 4th edition. IARC Press; 2010.

19. Royal College of Pathologists. Cancer database and tissue pathways. January 2018 [internet publication].Full text

20. Landry CS, Brock G, Scoggins CR, et al. A proposed staging system for rectal carcinoid tumors based on an analysis of 4701 patients. Surgery. 2008 Sep;144(3):460-6. Abstract

21. Rindi G, Arnold R, Bosman FT, et al. Nomenclature and classification of neuroendocrine neoplasms of the digestive system. In: Bosman FT, Hruban RH, Theise N, eds. WHO classification of tumors of the digestive system. 4th ed. Lyon: International Agency for Research on Cancer; 2010:13-4.

22. National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors [internet publication].Full text

23. Singh S, Asa SL, Dey C, et al. Diagnosis and management of gastrointestinal neuroendocrine tumors: an evidence-based Canadian consensus. Cancer Treat Rev. 2016 Jun;47:32-45.Full text  Abstract

24. Kunz PL, Reidy-Lagunes D, Anthony LB, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.Full text  Abstract

25. European Neuroendocrine Tumor Society. ENETS consensus guidelines for the diagnosis and treatment of neuroendocrine tumors. 2017 [internet publication].Full text

26. Riechelmann RP, Pereira AA, Rego JF, et al. Refractory carcinoid syndrome: a review of treatment options. Ther Adv Med Oncol. 2017 Feb;9(2):127-37.Full text  Abstract

27. Kaltsas G, Caplin M, Davies P, et al. NETS consensus guidelines for the standards of care inneuroendocrine tumors: pre- and perioperative therapy in patients with neuroendocrinetumors. Neuroendocrinology.2017;105(3):245-54.Full text  Abstract

28. Soga J, Yakuwa Y. Bronchopulmonary carcinoids: an analysis of 1,875 reported cases with special reference to a comparison between typical carcinoids and atypical varieties. Ann Thorac Cardiovasc Surg. 1999 Aug;5(4):211-9. Abstract

29. Chamberlain RS, Canes D, Brown KT, et al. Hepatic neuroendocrine metastases: does intervention alter outcomes? J Am Coll Surg. 2000 Apr;190(4):432-45. Abstract

30. Que FG, Nagorney DM, Batts KP, et al. Hepatic resection for metastatic neuroendocrine carcinomas. Am J Surg. 1995 Jan;169(1):36-42. Abstract

31. Hofland J, Herrera-Martínez AD, Zandee WT, et al. Management of carcinoid syndrome: a systematic review and meta-analysis. Endocr Relat Cancer. 2019 Mar;26(3):R145-56.Full text  Abstract

32. Lamberts SW, van der Lely AJ, de Herder WW, et al. Octreotide. N Engl J Med. 1996 Jan 25;334(4):246-54. Abstract

33. Shah T, Caplin M. Endocrine tumours of the gastrointestinal tract. Biotherapy for metastatic endocrine tumours. Best Pract Res Clin Gastroenterol. 2005 Aug;19(4):617-36. Abstract

34. O'Toole D, Ducreux M, Bommelaer G, et al. Treatment of carcinoid syndrome: a prospective crossover evaluation of lanreotide versus octreotide in terms of efficacy, patient acceptability, and tolerance. Cancer. 2000 Feb 15;88(4):770-6.Full text  Abstract

35. Oberg K, Kvols L, Caplin M, et al. Consensus report on the use of somatostatin analogs for the management of neuroendocrine tumors of the gastroenteropancreatic system. Ann Oncol. 2004 Jun;15(6):966-73.Full text  Abstract

36. Plockinger U, Wiedenmann B. Neuroendocrine tumors. Biotherapy. Best Pract Res Clin Endocrinol Metab. 2007 Mar;21(1):145-62. Abstract

37. Rinke A, Mueller HH, Schade-Brittinger C, et al; PROMID Study Group. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID Study Group. J Clin Oncol. 2009 Oct 1;27(28):4656-63.Full text  Abstract

38. Caplin ME, Pavel M, Ćwikła JB, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014 Jul 17;371(3):224-33.Full text  Abstract

39. Kulke MH, O'Dorisio T, Phan A, et al. Telotristat etiprate, a novel serotonin synthesis inhibitor, in patients with carcinoid syndrome and diarrhea not adequately controlled by octreotide. Endocr Relat Cancer. 2014 Oct;21(5):705-14.Full text  Abstract

40. Pavel M, Hörsch D, Caplin M, et al. Telotristat etiprate for carcinoid syndrome: a single-arm, multicenter trial. J Clin Endocrinol Metab. 2015 Apr;100(4):1511-9. Abstract

41. Kulke MH, Hörsch D, Caplin ME, et al. Telotristat ethyl, a tryptophan hydroxylase inhibitor for the treatment of carcinoid syndrome. J Clin Oncol. 2017 Jan;35(1):14-23.Full text  Abstract

42. Toumpanakis C, Meyer T, Caplin ME. Cytotoxic treatment including embolization/chemoembolization for neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab. 2007 Mar;21(1):131-44. Abstract

43. Eriksson BK, Larsson EG, Skogseid BM, et al. Liver embolizations of patients with malignant neuroendocrine gastrointestinal tumors. Cancer. 1998 Dec 1;83(11):2293-301. Abstract

44. Ruszniewski P, Rougier P, Roche A, et al. Hepatic arterial chemoembolization in patients with liver metastases of endocrine tumors: a prospective phase II study in 24 patients. Cancer. 1993 Apr 15;71(8):2624-30.Full text  Abstract

45. King J, Quinn R, Glenn DM, Janssen J, Tong D, Liaw W, Morris DL. Radioembolization with selective internal radiation microspheres for neuroendocrine liver metastases. Cancer. 2008 Sep 1;113(5):921-9.Full text  Abstract

46. Kwekkeboom DJ, Teunissen JJ, Bakker WH, et al. Radiolabeled somatostatin analog [177Lu-DOTA0,Tyr3]octreotate in patients with endocrine gastroenteropancreatic tumors. J Clin Oncol. 2005 Apr 20;23(12):2754-62.Full text  Abstract

47. Van Essen M, Krenning EP, de Jong M, et al. Peptide receptor radionuclide therapy with radiolabelled somatostatin analogues in patients with somatostatin receptor positive tumours. Acta Oncol. 2007;46(6):723-34. Abstract

48. Ruszniewski P. 177-Lu-Dotatate significantly improves progression-free survival in patients with midgut neuroendocrinetumours: results of the phase I NETTER-1 trial. Abstract 6LBA. European Cancer Congress; 2015.

49. Al-Toubah T, Morse B, Strosberg J. Capecitabine and temozolomide in advanced lung neuroendocrine neoplasms. Oncologist. 2020 Jan;25(1):e48-e52.Full text  Abstract

50. Ekeblad S, Sundin A, Janson ET, et al. Temozolomide as monotherapy is effective in treatment of advanced malignant neuroendocrine tumors. Clin Cancer Res. 2007 May 15;13(10):2986-91.Full text  Abstract

51. Frame J, Kelsen D, Kemeny N, et al. A phase II trial of streptozotocin and adriamycin in advanced APUD tumors. Am J Clin Oncol. 1988 Aug;11(4):490-5. Abstract

52. Moertel CG, Kvols LK, O'Connell MJ, et al. Treatment of neuroendocrine carcinomas with combined etoposide and cisplatin. Evidence of major therapeutic activity in the anaplastic variants of these neoplasms. Cancer. 1991 Jul 15;68(2):227-32. Abstract

53. Bajetta E, Rimassa L, Carnaghi C, et al. 5-Fluorouracil, dacarbazine, and epirubicin in the treatment of patients with neuroendocrine tumors. Cancer. 1998 Jul 15;83(2):372-8.Full text  Abstract

54. Halfdanarson TR, Strosberg JR, Tang L, et al. The North American Neuroendocrine Tumor Society consensus guidelines for surveillance and medical management of pancreatic neuroendocrine tumors. Pancreas. 2020 Aug;49(7):863-81.Full text  Abstract

55. Walter MA, Nesti C, Spanjol M, et al. Treatment for gastrointestinal and pancreatic neuroendocrine tumours: a network meta-analysis. Cochrane Database Syst Rev. 2021 Nov 25;11:CD013700.Full text  Abstract

56. Yao JC, Shah MH, Ito T, et al; RAD001 in Advanced Neuroendocrine Tumors, Third Trial (RADIANT-3) Study Group. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011 Feb 10;364(6):514-23.Full text  Abstract

57. Yao JC, Fazio N, Singh S, et al. Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (RADIANT-4): a randomised, placebo-controlled, phase 3 study. Lancet. 2016 Mar 5;387(10022):968-77. Abstract

58. Raymond E, Dahan L, Raoul JL, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011 Feb 10;364(6):501-13.Full text  Abstract

59. Oberg K, Eriksson B. The role of interferons in the management of carcinoid tumours. Br J Haematol. 1991 Oct;79(suppl 1):74-7. Abstract

60. Faiss S, Pape UF, Bohmig M, et al. Prospective, randomized, multicenter trial on the antiproliferative effect of lanreotide, interferon alfa, and their combination for therapy of metastatic neuroendocrine gastroenteropancreatic tumors--the International Lanreotide and Interferon Alfa Study Group. J Clin Oncol. 2003 Jul 15;21(14):2689-96. Abstract

61. Kulke MH, Mayer RJ. Carcinoid tumors. N Engl J Med. 1999 Mar 18;340(11):858-68. Abstract

62. Moertel CG, Kvols LK, Rubin J. A study of cyproheptadine in the treatment of metastatic carcinoid tumor and the malignant carcinoid syndrome. Cancer. 1991 Jan 1;67(1):33-6. Abstract

63. Eriksson B, Kloppel G, Krenning E, et al. Consensus guidelines for the management of patients with digestive neuroendocrine tumors: well-differentiated jejunal-ileal tumor/carcinoma. Neuroendocrinology. 2008;87(1):8-19. Abstract

64. Goede AC, Caplin ME, Winslet MC. Carcinoid tumour of the appendix. Br J Surg. 2003 Nov;90(11):1317-22. Abstract

65. Garland J, Buscombe JR, Bouvier C, et al. Sandostatin LAR (long-acting octreotide acetate) for malignant carcinoid syndrome: a 3-year experience. Aliment Pharmacol Ther. 2003 Feb;17(3):437-44.Full text  Abstract

66. Strosberg JR, Halfdanarson TR, Bellizzi AM, et al. The North American Neuroendocrine Tumor Society consensus guidelines for surveillance and medical management of midgut neuroendocrine tumors. Pancreas. 2017 Jul;46(6):707-14.Full text  Abstract

67. Howe JR, Cardona K, Fraker DL, et al. The surgical management of small bowel neuroendocrine tumors: consensus guidelines of the North American Neuroendocrine Tumor Society. Pancreas. 2017 Jul;46(6):715-31.Full text  Abstract

68. Singh S, Bergsland EK, Card CM, et al. Commonwealth Neuroendocrine Tumour Research Collaboration and the North American Neuroendocrine Tumor Society guidelines for the diagnosis and management of patients with lung neuroendocrine tumors: an international collaborative endorsement and update of the 2015 European Neuroendocrine Tumor Society expert consensus guidelines. J Thorac Oncol. 2020 Oct;15(10):1577-98.Full text  Abstract

69. Howe JR, Merchant NB, Conrad C, et al. The North American Neuroendocrine Tumor Society consensus paper on the surgical management of pancreatic neuroendocrine tumors. Pancreas. 2020 Jan;49(1):1-33.Full text  Abstract

70. Kerstrom G, Hellman P, Hessman O. Midgut carcinoid tumours: surgical treatment and prognosis. Best Pract Res Clin Gastroenterol. 2005 Oct;19(5):717-28. Abstract

71. Travis WD, Rush W, Flieder DB, et al. Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid. Am J Surg Pathol. 1998 Aug;22(8):934-44. Abstract

72. Ferguson MK, Landreneau RJ, Hazelrigg SR, et al. Long-term outcome after resection for bronchial carcinoid tumors. Eur J Cardiothorac Surg. 2000 Aug;18(2):156-61. Abstract

73. Hellman P, Lundstrom T, Ohrvall U, et al. Effect of surgery on the outcome of midgut carcinoid disease with lymph node and liver metastases. World J Surg. 2002 Aug;26(8):991-7. Abstract

74. Strosberg J, Gardner N, Kvols L. Survival and prognostic factor analysis of 146 metastatic neuroendocrine tumors of the mid-gut. Neuroendocrinology. 2009;89(4):471-6. Abstract

75. Ahmed A, Turner G, King B, et al. Midgut neuroendocrine tumours with liver metastases: results of the UKINETS study. Endocr Relat Cancer. 2009 Sep;16(3):885-94.Full text  Abstract

76. Maroun J, Kocha W, Kvols L, et al. Guidelines for the diagnosis and management of carcinoid tumours, part 1: the gastrointestinal tract. A statement from a Canadian National Carcinoid Expert Group. Curr Oncol. 2006;13:67-76.Full text  Abstract

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