Skip to main content
SpringerLink
Log in

Tumour Detection in Syndromic NET: Carcinoid Syndrome

  • Chapter
  • First Online:
Neuroendocrine Tumors in Real Life

Abstract

Many gastro-enteropancreatic neuroendocrine tumours (GEP-NETs) secrete biologically active substances and can present with distinct clinical syndromes related to their oversecretion. In particular, carcinoid syndrome (CS) is a compendium of clinical manifestations including secretory diarrhoea, flushing and less commonly wheezing and dyspnoea secondary to overproduction of such compounds, mainly serotonin. Carcinoid syndrome is almost exclusively derived from small intestinal NETs that have metastasized to the liver and may also be associated with extensive fibrosis of heart valves and the mesenterium. Although the diagnosis is usually delayed due to the lack of tumour-specific symptoms, measurement of specific tumour metabolites such as 5-hydroxyindoloacetic acid facilitates the diagnosis. Conventional radiology and functioning imaging modalities using specific and occasionally non-specific tracers are used to identify the extent of disease and also provide information regarding prognosis and the application of specific treatment. The development though of diagnostic and predictive biomarkers that would allow for individualized workup and selection of specific treatments remains a priority in the field.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

Bibliography

  1. Adaway JE, Dobson R, Walsh J, Cuthbertson DJ, Monaghan PJ, Trainer PJ, Valle JW, Keevil BG (2015) Serum and plasma 5-hydroxyindoleacetic acid as an alternative to 24-h urine 5-hydroxyindoleacetic acid measurement. Ann Clin Biochem 53(Pt 5):554–560. doi:10.1177/0004563215613109

    PubMed  Google Scholar 

  2. Albanus DR, Apitzsch J, Erdem Z, Erdem O, Verbung FA, Behrendt FF, Mottaghy FM, Heinzel A (2015) Clinical value of 68 DOTATATE -PET/CT compared to standard alone contrast enhanced CT for the detection of extra-hepatic metastases in patients with neuroendocrine tumors (NET). Eur J Radiol 84:1866–1872

    Article  CAS  PubMed  Google Scholar 

  3. Ambrosini V, Tomassetti P, Castellucci P, Campana D, Montini G, Rubello D, Nanni C, Rizzello A, Franchi R, Fanti S (2008) Comparison between 68Ga-DOTA-NOC and 18F-DOPA PET for the detection of gastro-entero-pancreatic and lung neuro-endocrine tumours. Eur J Nucl Med Mol Imaging 35:1431–1438

    Article  CAS  PubMed  Google Scholar 

  4. Ardill JE, Erikkson B (2003) The importance of the measurement of circulating markers in patients with neuroendocrine tumors of the pancreas and gut. Endocr Relat Cancer 10:459–462

    Article  CAS  PubMed  Google Scholar 

  5. Bailey AA, Debinski HS, Appleyard MN, Remedios ML, Hooper JE, Walsh AJ, Selby WS (2006) Diagnosis and outcome of small bowel tumors found by capsule endoscopy: a three center Australian experience. Am J Gastroenterol 101:2237–2243

    Article  PubMed  Google Scholar 

  6. Baudin E, Gigliotti A, Ducreux M et al (1998) Neuron-specific enolase and chromogranin A as markers of neuroendocrine tumors. Br J Cancer 78:1102–1107

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Bellutti M, Fry LC, Schmitt J, Seemann M, Klose S, Malfertheiner P, Monkemuller K (2009) Detection of neuroendocrine tumors of the small bowel by double balloon enteroscopy. Dig Dis Sci 54:1050–1058

    Article  PubMed  Google Scholar 

  8. Bhattacharyya S, Davar J, Dreyfus G, Caplin ME (2007) Carcinoid heart disease. Circulation 116:2860–2865

    Article  PubMed  Google Scholar 

  9. Dobson R, Cuthbertson DJ, Jones J, Valle JW, Keevil B, Chadwick C, Poston GP, Burgess MI (2014) Determination of the optimal echocardiographic scoring system to quantify carcinoid heart disease. Neuroendocrinology 99(2):85–93

    Google Scholar 

  10. Dorffel Y, Wermke W (2008) Neuroendocrine tumors: characterization with contrast-enhanced ultrasonography. Ultraschall Med 29:506–514

    Article  CAS  PubMed  Google Scholar 

  11. Gedde-Dahl M, Thiis-Evensen E, Myklebust Tjølsen A, Skrede Mordal K, Vatn M, Bergestuen DS (2013) Comparison of 24-h and overnight samples of urinary 5-hydroxyindoleacetic acid in patients with intestinal neuroendocrine tumors. Endocr Connect 2:50–54. doi:10.1530/EC-12-0077

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Janson ET, Holmberg L, Stridsberg M et al (1997) Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center. Ann Oncol 8:685–690

    Article  CAS  PubMed  Google Scholar 

  13. Kaltsas GA, Besser GM, Grossman AB (2004) The diagnosis and medical management of advanced neuroendocrine tumors. Endocr Rev 25:458–511. doi:10.1210/er.2003-0014

    Article  CAS  PubMed  Google Scholar 

  14. Kaltsas G, Rockall A, Papadogias D, Reznek R, Grossman AB (2004) Recent advances in radiological and radionuclide imaging and therapy of neuroendocrine tumours. Eur J Endocrinol 151:15–27

    Article  CAS  PubMed  Google Scholar 

  15. Kema IP, Meijer WG, Meiborg G, Ooms B, Willemse PH, de Vries EG (2001) Profiling of tryptophan- related plasma indoles in patients with carcinoid tumors by automated, on-line, solid-phase extraction and HPLC with fluorescence detection. Clin Chem 47:1811–1820

    CAS  PubMed  Google Scholar 

  16. Koopmans KP, de Vries EG, Kema IP, Elsinga PH, Neels OC, Sluiter WJ, van der Horst-Schrivers AN, Jager PL (2006) Staging of carcinoid tumors with 18F-DOPA PET: a prospective, diagnostic accuracy study. Lancet Oncol 7:728–734

    Article  CAS  PubMed  Google Scholar 

  17. Masselli G, Polettini E, Casciani E, Bertini L, Vecchioli A, Gualdi G (2009) Small-bowel neoplasms: prospective evaluation of MR enteroclysis. Radiology 251:743–750

    Article  PubMed  Google Scholar 

  18. Meijer WG, van der Veer E, Jager PL, van der Jagt EJ, Piers BA, Kema IP, de Vries EG, Willemse PH (2003) Bone metastases in carcinoid tumors: clinical features, imaging characteristics, and markers of bone metabolism. J Nucl Med 44:184–191

    PubMed  Google Scholar 

  19. Niederle B, Pape UF, Costa F, Gross D, Kelestimur F, Knigge U, Öberg K, Pavel M, Perren A, Toumpanakis C, O’Connor J, O’Toole D, Krenning E, Reed N, Kianmanesh R, Vienna Consensus Conference participants (2016) ENETS consensus guidelines update for neuroendocrine neoplasms of the jejunum and ileum. Neuroendocrinology 103(2):125–138. doi:10.1159/000443170. Epub 2016 Jan 12

    Article  CAS  PubMed  Google Scholar 

  20. O’Toole D, Grossman A, Gross D, Delle Fave G, Barkmanova J, O’Connor J, Pape UF, Plockinger U (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: biochemical markers. Neuroendocrinology 90:194–202

    Article  PubMed  Google Scholar 

  21. Panagiotidis E, Alshammari A, Michopoulou S, Skoura E, Naik K, Maragkoudakis E, Mohmaduvesh M, Al-Harbi M, Belda M, Caplin ME, Toumpanakis C, Bomanji J (2017) Comparison of the Impact of 68Ga-DOTATATE and 18F-FDG PET/CT on Clinical Management in Patients with Neuroendocrine Tumors. J Nucl Med 58(1):91–96

    Google Scholar 

  22. Papadogias D, Makras P, Kossivakis K, Kontogeorgos G, Piaditis G, Kaltsas G (2007) Carcinoid syndrome and carcinoid crisis secondary to a metastatic carcinoid tumor of the lung: a therapeutic challenge. Eur J Gastroenterol Hepatol 19(12):1154–1159

    Article  PubMed  Google Scholar 

  23. Prasad V, Ambrosini V, Hommann M et al (2010) Detection of unknown primary neuroendocrine tumors (CUP-NET) using (68) Ga-DOTA-NOC receptor PET/CT. Eur J Nucl Med Mol Imaging 37:67e77

    Article  Google Scholar 

  24. Schraml C, Schwenzer NF, Sperling O, Aschoff P, Lichy MP, Müller M, Brendle C, Werner MK, Claussen CD, Pfannenberg C (2013) Staging of neuroendocrine tumours: comparison of [68Ga]DOTATOC multiphase PET/CT and whole-body MRI. Cancer Imaging 5;13:63–72

    Google Scholar 

  25. Schreiter NF, Bartels AM, Froeling V, Steffen I, Pape UF, Beck A, Hamm B, Brenner W, Rottgen R (2014) Searching for primaries in patients with neuroendocrine tumors (NET) of unknown primary and clinically suspected NET: evaluation of Ga-68 DOTATOC PET/CT and in-111 DTPA octreotide SPECT/CT. Radiol Oncol 48(4):339–347

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Sundin A, Vullierme MP, Kaltsas G, Plöckinger U, all other Mallorca Consensus Conference participants (2009) ENETS consensus guidelines for the standards of care in neuroendocrine tumors: radiological examinations. Neuroendocrinology 90:167–183. doi:10.1159/000184855

    Article  CAS  PubMed  Google Scholar 

  27. Tomassetti P, Migliori M, Lalli S, Campana D, Tomassetti V, Corinaldesi R (2001) Epidemiology, clinical features and diagnosis of gastroenteropancreatic endocrine tumors. Ann Oncol 12(Suppl 2):S95–S99

    Article  PubMed  Google Scholar 

  28. Vinik A, Silva MP, Woltering EA, Go VL, Warner R, Caplin M (2009) Biochemical testing for neuroendocrine tumors. Pancreas 38(8):876–889

    Article  PubMed  Google Scholar 

  29. Zatelli MC, Torta M, Leon A et al (2007) Chromogranin A as a marker of neuroendocrine neoplasia: an Italian multicenter study. Endocr Relat Cancer 14:473–482

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. The Arden NET CoE, Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry, CV2 2DX, UK

    Georgios K. Dimitriadis

  2. Department of Pathophysiology, National & Kapodistrian University of Athens, Mikras Asias 75, 11527, Athens, Greece

    Gregory Kaltsas

  3. Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism, University Hospitals of Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry, CV2 2DX, UK

    Gregory Kaltsas

Authors
  1. Georgios K. Dimitriadis
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Gregory Kaltsas
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Gregory Kaltsas .

Editor information

Editors and Affiliations

  1. ENETs Center of Excellence for Neuroendocrine Tumours, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy

    Annamaria Colao

  2. ENETs Center of Excellence for Neuroendocrine Tumours, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy

    Antongiulio Faggiano

  3. Endocrine Oncology, Internal Medicine Department, Erasmus University, Rotterdam, Zuid-Holland, The Netherlands

    Wouter de Herder

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Cite this chapter

Dimitriadis, G.K., Kaltsas, G. (2018). Tumour Detection in Syndromic NET: Carcinoid Syndrome. In: Colao, A., Faggiano, A., de Herder, W. (eds) Neuroendocrine Tumors in Real Life. Springer, Cham. https://doi.org/10.1007/978-3-319-59024-0_10

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-59024-0_10

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-59022-6

  • Online ISBN: 978-3-319-59024-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation