Neuroendocrine Tumours: a Literature Review
https://doi.org/10.24060/2076-3093-2021-11-2-174-182
Abstract
Neuroendocrine tumours (NETs) are a heterogeneous group of malignant neoplasms with diverse morphology and nomenclature. Well-differentiated NETs were historically termed carcinoid tumours, which entailed abundant confusion and misclassification. Cross body-localised NETs have been described from the central nervous system, respiratory and gastrointestinal tracts, larynx, thyroid, skin, breast and urogenital system. The evidence on NET prevalence is diverse, with selected sources estimating a 0.5% rate among total malignancies diagnosed. Carcinoid syndrome is a known important associate of NETs. Its presence resulting from the amine and peptide hypersecretion often facilitates the NET diagnosis, and curative surgery becomes a treatment of choice, if technically feasible. Adjuvant therapy is ambiguous. When surgery is impractical due to a usually advanced NET at diagnosis, drug therapy is adopted to relief symptoms and control the disease.
About the Authors
K. V. MenshikovRussian Federation
Konstantin V. Menshikov — Cand. Sci. (Med.), Assoc. Prof., Department of Oncology with Courses of Oncology and Pathological Anatomy for Advanced Professional Education, Chemotherapy Unit
Ufa, Russian Federation
A. V. Sultanbaev
Russian Federation
Aleksandr V. Sultanbaev — Cand. Sci. (Med.), Outpatient Chemotherapy Unit
Ufa, Russian Federation
Sh. I. Musin
Russian Federation
Shamil I. Musin — Cand. Sci. (Med.), Surgery Unit No. 6
Ufa, Russian Federation
I. A. Menshikova
Russian Federation
Irina A. Menshikova — Cand. Sci. (Med.), Department of Biological Chemistry,
Ufa, Russian Federation
A. F. Nasretdinov
Russian Federation
Ainur F. Nasretdinov — Outpatient Chemotherapy Unit
Ufa, Russian Federation
N. I. Sultanbaeva
Russian Federation
Nadezhda I. Sultanbaeva — Anticancer Drug Therapy Unit
Ufa, Russian Federation
I. R. Shaykhutdinov
Russian Federation
Ilnur R. Shaykhutdinov — Dental Outpatient Clinic
Ufa, Russian Federation
References
1. Zandee W.T., de Herder W.W. The evolution of neuroendocrine tumor treatment reflected by ENETS Guidelines. Neuroendocrinology. 2018;106(4):357–65. DOI: 10.1159/000486096
2. Scalettar B.A., Jacobs C., Fulwiler A., Prahl L., Simon A., Hilken L., et al. Hindered submicron mobility and long-term storage of presynaptic dense-core granules revealed by single-particle tracking. Dev Neurobiol. 2012;72(9):1181–95. DOI: 10.1002/dneu.20984
3. Oronsky B., Ma P.C., Morgensztern D., Carter C.A. Nothing But NET: A Review of neuroendocrine tumors and carcinomas. Neoplasia. 2017;19(12):991–1002. DOI: 10.1016/j.neo.2017.09.002
4. Bosman F.T., Carneiro F., Hruban R.H., Theise N.D. Classification of tumours of the digestive system. 4th ed. Geneva: WHO Press; 2010.
5. Kim J.Y., Hong S.M., Ro J.Y. Recent updates on grading and classification of neuroendocrine tumors. Ann Diagn Pathol. 2017;29:11–6. DOI: 10.1016/j.anndiagpath.2017.04.005
6. Rossi G., Bertero L., Marchiò C., Papotti M. Molecular alterations of neuroendocrine tumours of the lung. Histopathology. 2018;72(1):142–52. DOI: 10.1111/his.13394
7. Taal B.G., Visser O. Epidemiology of neuroendocrine tumours. Neuroendocrinology. 2004;80 Suppl 1:3–7. DOI: 10.1159/000080731
8. Cives M., Strosberg J.R. Gastroenteropancreatic neuroendocrine tumors. CA Cancer J Clin. 2018;68(6):471–87. DOI: 10.3322/caac.21493
9. Dasari A., Shen C., Halperin D., Zhao B., Zhou S., Xu Y., et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017;3(10):1335–42. DOI: 10.1001/jamaoncol.2017.0589
10. Abstracts Presented at the 7th Annual Meeting of the North American NeuroEndocrine Tumor Society, October 10–11, 2014, Nashville. Tennessee Pancreas J. 2015;44(2);347–62.
11. Avcu S., Ozen O., Bulut M.D., Bora A. Hepatic metastases of primary jejunal carcinoid tumor: A case report with radiological findings. N Am J Med Sci. 2009;1(6):305–8. PMID: 22666712
12. Fink G., Krelbaum T., Yellin A., Bendayan D., Saute M., Glazer M., et al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest. 2001;119(6):1647–51. DOI: 10.1378/chest.119.6.1647
13. Kaprin A.D., Starinsky V.V., Shakhzadova A.O. (eds) State of cancer care for population in Russia in 2019. Moscow: National Medical Research Radiological Centre of the Ministry of Health of the Russian Federation, 2020 (In Russ.).
14. Klimstra D.S. Pathology reporting of neuroendocrine tumors: essential elements for accurate diagnosis, classification, and staging. Semin Oncol. 2013;40(1):23–36. DOI: 10.1053/j.seminoncol.2012.11.001
15. Bosman F.T., Carneim F., Hruban R.H., Theise N.D. WHO classification of tumours of the digestive system. Lyon: IARC Press; 2010.
16. Modlin I.M., Shapiro M.D., Kidd M. Siegfried Oberndorfer: origins and perspectives of carcinoid tumors. Hum Pathol. 2004;35(12):1440–51. DOI: 10.1016/j.humpath.2004.09.018
17. Rosai J. The origin of neuroendocrine tumors and the neural crest saga. Mod Pathol. 2011;24 Suppl 2:S53–7. DOI: 10.1038/modpathol.2010.166
18. Williams E., Sandler M. The classification of carcinoid tumours. Lancet. 1963;1(7275):238–9. DOI: 10.1016/s0140-6736(63)90951-6
19. Soga J. The term “carcinoid” is a misnomer: the evidence based on local invasion. J Exp Clin Cancer Res. 2009;28(1):15. DOI: 10.1186/1756-9966-28-15
20. Creutzfeldt W. Carcinoid tumors: development of our knowledge. World J Surg. 1996;20(2):126–31. DOI: 10.1007/s002689900020
21. Chang S., Choi D., Lee S.J., Lee W.J., Park M.H., Kim S.W., et al. Neuroendocrine neoplasms of the gastrointestinal tract: classification, pathologic basis, and imaging features. Radiographics. 2007;27(6):1667–79. DOI: 10.1148/rg.276075001
22. Howe J.R. Carcinoid Tumors: Past, Present, and Future. Indian J Surg Oncol. 2020;11(2):182–7. DOI: 10.1007/s13193-020-01079-6
23. Pusceddu S., Catena L., Valente M., Buzzoni R., Formisano B., Del Vecchio M., et al. Long-term follow up of patients affected by pulmonary carcinoid at the Istituto Nazionale Tumori of Milan: a retrospective analysis. J Thorac Dis. 2010;2(1):16–20. PMID: 22263011
24. Klöppel G. Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms. Endocr Relat Cancer. 2011;18 (Suppl 1):S1–16. DOI: 10.1530/ERC-11-0013
25. Travis W.D. The concept of pulmonary neuroendocrine tumours. In: Travis W.D., Brambilla E., Muller-Hermelink H.K., Harris C.C., (eds.) Pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004.
26. Kulke M.H., Shah M.H., Benson A.B. 3rd, Bergsland E., Berlin J.D., Blaszkowsky L.S., et al. Neuroendocrine tumors, version 1.2015. J Natl Compr Canc Netw. 2015;13(1):78–108. DOI: 10.6004/jnccn.2015.0011
27. Gut P., Komarowska H., Czarnywojtek A., Waligórska-Stachura J., Bączyk M., Ziemnicka K., et al. Familial syndromes associated with neuroendocrine tumours. Contemp Oncol (Pozn). 2015;19(3):176–83. DOI: 10.5114/wo.2015.52710
28. Di Domenico A., Wiedmer T., Marinoni I., Perren A. Genetic and epigenetic drivers of neuroendocrine tumours (NET). Endocr Relat Cancer. 2017;24(9):R315–34. DOI: 10.1530/ERC-17-0012
29. Brandi M.L., Gagel R.F., Angeli A., Bilezikian J.P., Beck-Peccoz P., Bordi C., et al. Guidelines for diagnosis and therapy of MEN type 1 and type 2. J Clin Endocrinol Metab. 2001;86(12):5658–71. DOI: 10.1210/jcem.86.12.8070
30. Jiao Y., Shi C., Edil B.H., de Wilde R.F., Klimstra D.S., Maitra A., et al. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011;331(6021):1199–203. DOI: 10.1126/science.1200609
31. Bousquet C., Lasfargues C., Chalabi M., Billah S.M., Susini C., Vezzosi D., et al. Clinical review: current scientific rationale for the use of somatostatin analogs and mTOR inhibitors in neuroendocrine tumor therapy. J Clin Endocrinol Metab. 2012;97(3):727–37. DOI: 10.1210/jc.2011-2088
32. Speisky D., Duces A., Bièche I., Rebours V., Hammel P., Sauvanet A., et al. Molecular profiling of pancreatic neuroendocrine tumors in sporadic and Von Hippel-Lindau patients. Clin Cancer Res. 2012;18(10):2838–49. DOI: 10.1158/1078-0432.CCR-11-2759
33. Vollbrecht C., Werner R., Walter R.F., Christoph D.C., Heukamp L.C., Peifer M., et al. Mutational analysis of pulmonary tumours with neuroendocrine features using targeted massive parallel sequencing: a comparison of a neglected tumour group. Br J Cancer. 2015;113(12):1704–11. DOI: 10.1038/bjc.2015.397
34. Lips C.J., Lentjes E.G., Höppener J.W. The spectrum of carcinoid tumours and carcinoid syndromes. Ann Clin Biochem. 2003;40(Pt 6):612–27. DOI: 10.1258/000456303770367207
35. Wolin E.M., Benson Iii A.B. Systemic treatment options for carcinoid syndrome: a systematic review. Oncology. 2019;96(6):273–89. DOI: 10.1159/000499049
36. Gustafsson B.I., Tommeras K., Nordrum I., Loennechen J.P., Brunsvik A., Solligård E., et al. Long-term serotonin administration induces heart valve disease in rats. Circulation. 2005;111(12):1517–22. DOI: 10.1161/01.CIR.0000159356.42064.48
37. de Vries H., Verschueren R.C., Willemse P.H., Kema I.P., de Vries E.G. Diagnostic, surgical and medical aspect of the midgut carcinoids. Cancer Treat Rev. 2002;28(1):11–25. DOI: 10.1053/ctrv.2001.0239
38. Fanciulli G., Ruggeri R.M., Grossrubatscher E., Calzo F.L., Wood T.D., Faggiano A., et al. Serotonin pathway in carcinoid syndrome: clinical, diagnostic, prognostic and therapeutic implications. Rev Endocr Metab Disord. 2020;21(4):599–612. DOI: 10.1007/s11154-020-09547-8
39. Ramage J.K., Ahmed A., Ardill J., Bax N., Breen D.J., Caplin M.E., et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2012;61(1):6–32. DOI: 10.1136/gutjnl-2011-300831
40. Graham G.W., Unger B.P., Coursin D.B. Perioperative management of selected endocrine disorders. Int Anesthesiol Clin. 2000;38(4):31–67. DOI: 10.1097/00004311-200010000-00004
41. Reichman O., Sobel J.D. Vulvovaginal pellagra and lichen sclerosus complicating carcinoid syndrome. Obstet Gynecol. 2009;113(2 Pt2):543–5. DOI: 10.1097/AOG.0b013e318191bb51
42. Kerström G., Hellman P., Hessman O. Midgut carcinoid tumours: surgical treatment and prognosis. Best Pract Res Clin Gastroenterol. 2005;19(5):717–28. DOI: 10.1016/j.bpg.2005.05.005
43. Coruh A.G., Kul M., Kuru Oz D., Yenigun B., Cansız Ersoz C., Ozalp Ateş F., et al. Is it possible to discriminate pulmonary carcinoids from hamartomas based on CT features? Clin Imaging. 2020;62:49–56. DOI: 10.1016/j.clinimag.2020.02.001
44. Chong S., Lee K.S., Chung M.J., Han J., Kwon O.J., Kim T.S. Neuroendocrine tumors of the lung: clinical, pathologic, and imaging findings. Radiographics. 2006;26(1):41–57; discussion 57–8. DOI: 10.1148/rg.261055057
45. Caplin M.E., Baudin E., Ferolla P., Filosso P., Garcia-Yuste M., Lim E., et al. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. Ann Oncol. 2015;26(8):1604–20. DOI: 10.1093/annonc/mdv041
46. Salyers W.J., Vega K.J., Munoz J.C., Trotman B.W., Tanev S.S. Neuroendocrine tumors of the gastrointestinal tract: Case reports and literature review. World J Gastrointest Oncol. 2014;6(8):301–10. DOI: 10.4251/wjgo.v6.i8.301
47. Kulke M.H. Somatostatin analogues in neuroendocrine tumors. J Natl Compr Canc Netw. 2016;14(3):241–2. DOI: 10.6004/jnccn.2016.0029
48. Kulkarni R.S., Anand A.S., Parikh S.K., Panchal H.P., Patel A.A., Mehta D.P., et al. Clinical and epidemiological profile of neuroendocrine tumors: An experience from a regional cancer center from Western India. South Asian J Cancer. 2019;8(3):198–202. DOI: 10.4103/sajc.sajc_364_18
49. Kulke M.H. Clinical presentation and management of carcinoid tumors. Hematol Oncol Clin North Am. 2007;21(3):433–55; vii–viii. DOI: 10.1016/j.hoc.2007.04.004
50. Chou W.C., Chen J.S., Hung Y.S., Hsu J.T., Chen T.C., Sun C.F., et al. Plasma chromogranin A levels predict survival and tumor response in patients with advanced gastroenteropancreatic neuroendocrine tumors. Anticancer Res. 2014;34(10):5661–9. PMID: 25275071
51. Oberg K. Circulating biomarkers in gastroenteropancreatic neuroendocrine tumours. Endocr Relat Cancer. 2011;18 (Suppl 1):S17–25. DOI: 10.1530/ERC-10-0280
52. Oronsky B., Ma P.C., Morgensztern D., Carter C.A. Nothing but NET: a review of neuroendocrine tumors and carcinomas. Neoplasia. 2017;19(12):991–1002. DOI: 10.1016/j.neo.2017.09.002
53. Kulke M.H., Siu L.L., Tepper J.E., Fisher G., Jaffe D., Haller D.G., et al. Future directions in the treatment of neuroendocrine tumors: consensus report of the National Cancer Institute Neuroendocrine Tumor clinical trials planning meeting. J Clin Oncol. 2011;29(7):934–43. DOI: 10.1200/JCO.2010.33.2056
54. Oberg K.E. The management of neuroendocrine tumours: current and future medical therapy options. Clin Oncol (R Coll Radiol). 2012;24(4):282–93. DOI: 10.1016/j.clon.2011.08.006
55. Frilling A., Clift A.K. Therapeutic strategies for neuroendocrine liver metastases. Cancer. 2015;121(8):1172–86. DOI: 10.1002/cncr.28760
56. Faggiano A., Lo Calzo F., Pizza G., Modica R., Colao A. The safety of available treatments options for neuroendocrine tumors. Expert Opin Drug Saf. 2017;16(10):1149–61. DOI: 10.1080/14740338.2017.1354984
Review
For citations:
Menshikov K.V., Sultanbaev A.V., Musin Sh.I., Menshikova I.A., Nasretdinov A.F., Sultanbaeva N.I., Shaykhutdinov I.R. Neuroendocrine Tumours: a Literature Review. Creative surgery and oncology. 2021;11(2):174-182. (In Russ.) https://doi.org/10.24060/2076-3093-2021-11-2-174-182