Carcinoid syndroma – diagnosis and management
Authors:
Soňa Kiňová; Michal Koreň
Authors‘ workplace:
I. interná klinika LF UK a UN Bratislava
Published in:
Vnitř Lék 2021; 67(5): 310-314
Category:
Review Articles
Overview
Neuroendocrine tumors (NETs, originally termed “carcinoids”) create a relatively rare group of neoplasms with an approximate incidence rate of 5 to 8 cases per 10 000 persons. NETs predominantly demonstrate indolent disease biology for many years. They become symptomatic when they are large enough or when they metastasize to the liver or the lungs, bones, or other sites. Roughly 30% to 40% of subjects with NETs develop carcinoid syndrome. Signs and symptoms of carcinoid syndrome are bronchospasm, flushing, diarrhea and cramping, cyanosis and pellagra. White plaque-like deposits on the endocardial surface of heart structures are characteristic for carcinoid heart disease. The treatment of patients with carcinoid syndrome is multi-faceted due to the necessity to manage simultaneously the systemic cancer disease as well as the signs of carcinoid syndrome and includes resection or debulking of tumor mass, biological treatment with somatostatin analogues and peptide receptor radionuclide treatment.
Keywords:
neuroendocrine tumors – biological treatment – carcinoid syndroma – somatostatin analogues – peptide receptor radionuclide treatment
Sources
1. Lawrence B, Gustafsson BI et al. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin North Am. 2011; 40(1): 1–18.
2. Boyar Cetinkaya R, Aagnes B, Thiis-Evensen E et al. Trends in Incidence of Neuroendocrine Neoplasms in Norway: A Report of 16,075 Cases from 1993 through 2010. Neuroendocrinology. 2017; 104(1): 1–10.
3. Hauso O, Gustafsson BI, Kidd M et al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer. 2008; 113(10): 2655–2664.
4. 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; 3(10): 1335–1342.
5. Kieran AC, Kidd M, Bodeic L et al. Neuroendocrine Neoplasms of the Small Bowel and Pancreas. Neuroendocrinology 2020; 110: 444–476.
6. Lubarsch O. Uber den primären Krebs des Ileum, nebst Bemerkungen úber das gleichzeitige Vorkommen von Krebs und Tuberkulose. Virchoows Arch. 1888; (111): 280–317.
7. Ranson WB. A case of primary carcinoma of the ileum. Lancet II, 1890: 1020–1022.
8. Gosset A, Masson P. Tumour endocrines de l appendice. Presse Med., 1914; (22): 237–240.
9. Kiňová S, Bulas J, Murín J et al. Postihnutie endokardu pri malígnom karcinoide. Kardiológia, 2001; (10)2: 79–82.
10. Feldman JM. Carcinoid tumors and the carcinoid syndrome. Curr. Probl. Surg., 1989 (26): 835–858.
11. Glasberg SM, Grossman AB, Gross DJ. Carcinoid Hearth Disease – From Pathophysiology to Treatment. “Something in the Way it moves”. Neuroendocrinology 2015; (101): 263–273.
12. Memon MA, Nelson H. Gastrointestinal carcinoid tumors. Current management strategies. Dis. Colon. Rectum, 1997; 9(vol. 40): 1101–1118.
13. Connolly HM, Hartzell VS, Mullany JCh et al. Carcinoid heart disease: Impact of Pulmonary valve replacement in right ventricular function and remodeling. Circulation, 2002; (106) suppl. I: 51–56.
14. Moyssakis IE, Rallidis LS, Guida GF. Incidence and evolution of carcinoid syndrome in the heart. J. heart Valve Dis., 1997; 6(6): 625–630.
15. 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; 88(4): 770–776.
16. Oberg K, Couvelard A, Delle Fave G et al. ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Biochemical Markers. Neuroendocrinology 2017; 105: 201–211.
17. Sundin A, Arnold B, Baudin E et al. ENETs Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Radiological, Nuclear Medicine and Hybride Methods. Neuroendocrinology 2017; 105: 212–244.
18. Pavel M, Valle JW, Eriksson B et al. The ENETs Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy – Biotherapy and Novel Targeted Agents. Neuroendocrinology 2017; 105: 266–280.
19. Carbonero G, Rinke RA, Valle JW et al. ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Neoplasms: Systemic Therapy – Chemotherapy Neuroendocrinology 2017; 105: 281–294.
20. Davar J et al. Diagnosing and Managing Carcinoid hearth disease in patients with Neuroendocrine tumors. JACC, 2017.
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Diabetology Endocrinology Internal medicineArticle was published in
Internal Medicine
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