Abstract
Neuroendocrine tumours (NETs) are generally slow-growing cancers with a rising incidence. Public Health England recorded 8726 neuroendocrine neoplasms (3978 NETs) over 2 years (2013–2014), yielding an overall incidence rate of 8 per 100,000 persons. NETs are often diagnosed incidentally, but most are discovered following investigation of symptoms. NETs commonly arise in the gastrointestinal tract, pancreas or lung, although primary site could be anywhere in the body. Local and distal metastases are common at diagnosis. Gastrointestinal NETs can cause life-threatening peritoneal and cardiac valve fibrosis.
Diagnosis of NETs relies on clinical features, biochemical assessment, imaging and histopathology. They are histologically graded from 1 to 3 according to the WHO 2010 classification system. Radiological assessment relies on anatomic and functional imaging.
Early diagnosis of NETs is challenging due to the rare nature of the disease as well as their presentation with non-specific symptoms. Patients are commonly diagnosed late, when metastatic disease is present and cure is not possible. There is a need for earlier diagnosis to increase treatment options and improve prognosis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Incidence and survival in neuroendocrine tumours and neuroendocrine carcinomas (NETs/NECs) in England, 2013–2014. Public Health England. 2016.
Oberg K, Castellano D. Current knowledge on diagnosis and staging of neuroendocrine tumors. Cancer Metastasis Rev. 2011;30(Suppl 1):3–7.
Jernman J, Välimäki MJ, Louhimo J, Haglund C, Arola J. The Novel WHO 2010 classification for gastrointestinal neuroendocrine tumour correlates well with the metastatic potential of rectal neuroendocrine tumours. Neuroendocrinology. 2012;95(4):317–24.
Ramage JK, Ahmed A, Ardill J, Bax N, Breen DJ, Caplin ME, et al. Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut. 2012;61(1):6–32.
Xavier S, Rosa B, Cotter J. Small bowel neuroendocrine tumors: from pathophysiology to clinical approach. World J Gastrointest Pathophysiol. 2016;7(1):117–24.
Debas HT, Mulvihill SJ. Neuroendocrine gut neoplasms. Important lessons from uncommon tumors. Arch Surg. 1994;129(9):965–71.
Kytölä S, Nord B, Elder EE, Carling T, Kjellman M, Cedermark B, et al. Alterations of the SDHD gene locus in midgut carcinoids, Merkel cell carcinomas, pheochromocytomas, and abdominal paragangliomas. Genes Chromosomes Cancer. 2002 Jul;34(3):325–32.
Hassan MM, Phan A, Li D, Dagohoy CG, Leary C, Yao JC. Risk factors associated with neuroendocrine tumors: A U.S.-based case-control study. Int J Cancer. 2008;123(4):867–73.
Saha S, Hoda S, Godfrey R, Sutherland C, Raybon K. Carcinoid tumors of the gastrointestinal tract: a 44-year experience. South Med J. 1989;82(12):1501–5.
Delle Fave G, O’Toole D, Sundin A, Taal B, Ferolla P, Ramage JK, et al. ENETS consensus guidelines update for gastroduodenal neuroendocrine neoplasms. Neuroendocrinology. 2016;103(2):119–24.
Li TT, Qiu F, Qian ZR, Wan J, Qi XK, Wu BY. Classification, clinicopathologic features and treatment of gastric neuroendocrine tumors. World J Gastroenterol. 2014;20(1):118–25.
Hoffmann KM, Furukawa M, Jensen RT. Duodenal neuroendocrine tumors: Classification, functional syndromes, diagnosis and medical treatment. Best Pract Res Clin Gastroenterol. 2005;19(5):675–97.
Niederle B, Pape UF, Costa F, Gross D, Kelestimur F, Knigge U, et al. ENETS consensus guidelines update for neuroendocrine neoplasms of the jejunum and ileum. Neuroendocrinology. 2016;103(2):125–38.
Ramage JK, Goretzki PE, Manfredi R, Komminoth P, Ferone D, Hydrel R, et al. Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated colon and rectum tumour/carcinoma. Neuroendocrinology. 2008;87(1):31–9.
Caplin ME, 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.
Bhattacharyya S, Davar J, Dreyfus G, Caplin ME. Carcinoid heart disease. Circulation. 2007;116(24):2860–5.
Bhattacharyya S, Burke M, Caplin ME, Davar J. Utility of 3D transoesophageal echocardiography for the assessment of tricuspid and pulmonary valves in carcinoid heart disease. Eur J Echocardiogr. 2011;12(1):E4.
Basuroy R, Srirajskanthan R, Ramage JK. Neuroendocrine tumors. Gastroenterol Clin N Am. 2016;45(3):487–507.
Dde Herder WW, Kwekkeboom DJ, Valkema R, Feelders RA, van Aken MO, Lamberts SW, et al. Neuroendocrine tumors and somatostatin: imaging techniques. J Endocrinol Invest. 2005;28(11 Suppl International):132–6.
Gabriel M, Decristoforo C, Kendler D, Dobrozemsky G, Heute D, Uprimny C, et al. 68Ga-DOTA-Tyr3-octreotide PET in neuroendocrine tumors: comparison with somatostatin receptor scintigraphy and CT. J Nucl Med. 2007;48(4):508–18.
Acknowledgements
We would like to thank Dr. Salil Karkhanis for his contribution in writing this chapter.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2019 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Sagar, V.M., Elshafie, M., Shah, T. (2019). Making the Diagnosis of Neuroendocrine Tumour Disease. In: Cross, T., Palmer, D. (eds) Liver Cancers. Springer, Cham. https://doi.org/10.1007/978-3-319-92216-4_19
Download citation
DOI: https://doi.org/10.1007/978-3-319-92216-4_19
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-92215-7
Online ISBN: 978-3-319-92216-4
eBook Packages: MedicineMedicine (R0)