Carcinoid syndrome

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

North American Neuroendocrine Tumor Society. NANETS guidelines​. 2022 [internet publication].Full text

1. WHO Classification of Tumours Editorial Board. WHO classification of tumours: endocrine and neuroendocrine tumours. 5th ed. Lyon:International Agency for Research on Cancer;2025.​

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

3. 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

4. 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

5. Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017 Oct 1;3(10):1335-42.Full text  Abstract

6. 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

7. White BE, Rous B, Chandrakumaran K, et al. Incidence and survival of neuroendocrine neoplasia in England 1995-2018: a retrospective, population-based study. Lancet Reg Health Eur. 2022 Dec;23:100510.Full text  Abstract

8. 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

9. 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

10. 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

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

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

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

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

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

16. North American Neuroendocrine Tumor Society. NANETS guidelines​. 2022 [internet publication].Full text

17. 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.

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

19. 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

20. WHO Classification of Tumours Editorial Board. WHO classification of tumours: digestive system tumours. 5th ed. Lyon:International Agency for Research on Cancer;2019.​

21. 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

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

23. 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

24. 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

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

26. 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

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

28. 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

29. 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

30. 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

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

32. 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

33. 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

34. 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

35. 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

36. 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

37. 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

38. 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

39. 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

40. 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

41. 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

42. 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

43. Strosberg J, El-Haddad G, Wolin E, et al. Phase 3 trial of (177)Lu-Dotatate for midgut neuroendocrine tumors. N Engl J Med. 2017 Jan 12;376(2):125-35.Full text  Abstract

44. Strosberg JR, Caplin ME, Kunz PL, et al. (177)Lu-Dotatate plus long-acting octreotide versus high‑dose long-acting octreotide in patients with midgut neuroendocrine tumours (NETTER-1): final overall survival and long-term safety results from an open-label, randomised, controlled, phase 3 trial. Lancet Oncol. 2021 Dec;22(12):1752-63. Abstract

45. Singh S, Halperin D, Myrehaug S, et al. [(177)Lu]Lu-DOTA-TATE plus long-acting octreotide versus high‑dose long-acting octreotide for the treatment of newly diagnosed, advanced grade 2-3, well-differentiated, gastroenteropancreatic neuroendocrine tumours (NETTER-2): an open-label, randomised, phase 3 study. Lancet. 2024 Jun 29;403(10446):2807-17. Abstract

46. 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

47. 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

48. 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

49. 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

50. 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

51. 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

52. 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

53. 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

54. 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

55. 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

56. 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

57. 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

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

59. 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

60. 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

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

62. 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

63. Chan JA, Geyer S, Zemla T, et al. Phase 3 trial of cabozantinib to treat advanced neuroendocrine tumors. N Engl J Med. 2025 Feb 13;392(7):653-65.Full text  Abstract

64. 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

65. 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

66. 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

67. 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

68. 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

69. 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

70. Grozinsky-Glasberg S, Davar J, Hofland J, et al. European Neuroendocrine Tumor Society (ENETS) 2022 guidance paper for carcinoid syndrome and carcinoid heart disease. J Neuroendocrinol. 2022 Jul;34(7):e13146.Full text

71. 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 Apr;13(2):67-76. Abstract