Skip to main content

Tumour Staging: Ileum

  • Chapter
  • First Online:
Neuroendocrine Tumors in Real Life

Abstract

Ileal neuroendocrine tumours are one of the most common gastro-enteropancreatic neuroendocrine tumours (GEP-NETs), making up to 28% of all GEP-NETs. Small bowel NETs are the most common type of small bowel tumour; 45% of these lesions occur in the distal ileum. Small bowel neuroendocrine tumours commonly present in the sixth to seventh decade; however, they can occur at any time of life. In up to 30% of cases, these lesions are multiple, with some case series reporting this to be as high as 40%. When diagnosed, ileal NETs are frequently larger than 2 cm and have spread to regional lymph nodes.

In this article, we will review the different staging methods of GEP-NETs and incorporate the tumour, node and metastases (TNM) staging systems developed by the European Neuroendocrine Tumour Society (ENETS) and American Joint committee on Cancer (AJCC). The importance of radiology in staging will be discussed extensively, including the difficulties with each resource and the challenges in accurately staging and locating the primary site of disease. We will look at the role of histology in the grading of tumours, with focus on the mitotic and proliferation indices.

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

Access this chapter

Institutional subscriptions

Bibliography

  1. Pinchot SN, Holen K, Sippel RS et al (2008) Carcinoid tumors. Oncologist 13:1255–1269

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Yamaguchi T, Manabe N, Tanaka S, Fukumoto A, Shimamoto M, Nakao M et al (2005) Multiple carcinoid tumours of the ileum preoperatively diagnosed by enteroscopy with the double-balloon technique. Gastrointest Endosc 62:315–318

    Article  PubMed  Google Scholar 

  3. Klöppel G (2007) Tumour biology and histopathology of neuroendocrine tumours. Best Pract Res Clin Endocrinol Metab 21(1):15–31

    Article  PubMed  Google Scholar 

  4. Geijer H, Breimer LH (2013) Somatostatin receptor PET/CT in neuroendocrine tumours: update on systematic review and meta-analysis. Eur J Nucl Med Mol Imaging 40(11):1770–1780

    Article  CAS  PubMed  Google Scholar 

  5. Kwee TC, Basu S, Saboury B, Ambrosini V, Torigian DA, Alavi A (2011) A new dimension of FDG-PET interpretation: assessment of tumor biology. Eur J Nucl Med Mol Imaging 38:1158–1170

    Article  PubMed  Google Scholar 

  6. Nilica B et al (2016) Direct comparison of 68Ga-DOTA-TOC and 18F-FDG PET/CT in the follow-up of patients with neuroendocrine tumour treated with the first full peptide receptor radionuclide therapy cycle. Eur J Nucl Med Mol Imaging 43:1585–1592

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  7. Kamaoui I, De-Luca V, Ficarelli S, Mennesson N, Lombard-Bohas C, Pilleul F (2010) Value of CT enteroclysis in suspected small-bowel carcinoid tumors. AJR Am J Roentgenol 194:629–633

    Article  PubMed  Google Scholar 

  8. Boudreaux JP, Klimstra DS, Hassan MM et al (2010) The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: well-differentiated neuroendocrine tumors of the Jejunum, Ileum, Appendix, and Cecum. North American Neuroendocrine Tumor Society (NANETS). Pancreas 39:753–766

    Article  PubMed  Google Scholar 

  9. Dromain C, de Baere T, Baudin E et al (2003) MR imaging of hepatic metastases caused by neuroendocrine tumors: comparing four techniques. Am J Roentgenol 180(1):121–128

    Article  Google Scholar 

  10. Debray MP, Geoffroy O, Laissy JP et al (2001) Imaging appearances of metastases from neuroendocrine tumours of the pancreas. Br J Radiol 74:1065–1070

    Article  CAS  PubMed  Google Scholar 

  11. Öberg K, Knigge U, Kwekkeboom D, Perren A (2012) Neuroendocrine Gastroenteropancreatic Tumours: ESMO clinical practice guidelines. Ann Oncol 23(Suppl 7):vii124–vii130

    PubMed  Google Scholar 

  12. Rockall AG, Reznek RH (2007) Imaging of neuroendocrine tumours (CT/MR/US). Best Pract Res Clin Endocrinol Metab 21:43–68

    Article  CAS  PubMed  Google Scholar 

  13. Sundin A, Vullierme MP, Kaltsas G et al (2009) ENETS consensus guidelines for the standards of Care in Neuroendocrine Tumors: radiological examinations. Neuroendocrinol 90:167–183

    Article  CAS  Google Scholar 

  14. Koopmans KP, Neels OC, Kema IP et al (2008) Improved staging of patients with carcinoid and islet cell tumors with 18F-dihydroxy-phenyl-alanine and 11C-5-hydroxy-tryptophan positron emission tomography. J Clin Oncol 26:1489–1495

    Article  PubMed  Google Scholar 

  15. Binderup T, Knigge U, Loft A et al (2010) 18F-fluorodeoxyglucose positron emission tomography predicts survival of patients with neuroendocrine tumors. Clin Cancer Res 16:978–985

    Article  CAS  PubMed  Google Scholar 

  16. Cave D, Legnani P, de Ranchis R et al (2005) ICCE consensus for capsule retention. Endoscopy 37:1065–1067

    Article  CAS  PubMed  Google Scholar 

  17. Cheon JH, Kim YS, Lee IS et al (2007) Can we predict spontaneous capsule passage after retention? A nationwide study to evaluate the incidence and clinical outcomes of capsule retention. Endoscopy 39:1046–1052

    Article  CAS  PubMed  Google Scholar 

  18. Barkin J, Friedman S (2002) Wireless capsule endoscopy requiring surgical intervention: the world’s experience. Am J Gastroenterol 97:A907

    Article  Google Scholar 

  19. Römmele C, Brueckner J, Messmann H, Gölder SK (2016) Clinical experience with the PillCam patency capsule prior to video capsule endoscopy: a real-world experience. Gastroenterol Res Pract 2016:9657053

    Article  PubMed  PubMed Central  Google Scholar 

  20. Sugimoto E, Lorelius LE, Eriksson B, Oberg K (1995) Midgut carcinoid tumours. CT appearance Acta Radiol 36:367–371

    Article  CAS  PubMed  Google Scholar 

  21. Wiener-Carrillo I, González-Alvarado C, Cervantes-Valladolid M, Echaverry-Navarrete D, Zubieta-O’Farrill G, Gudiño-Chávez A (2014) Intussusception secondary to a carcinoid tumor in an adult patient. Int J Surg Case Rep 5(5):265–267

    Article  PubMed  PubMed Central  Google Scholar 

  22. Paulson EK, McDermott VG, Keogan MT et al (1998) Carcinoid metastases to the liver: role of triple-phase helical CT. Radiol 206:143–150

    Article  CAS  Google Scholar 

  23. GoreRM BJW, Mehta UK, Newmark GM, Yaghmai V (2005) GI carcinoid tumours: appearance of the primary and detecting metastases. Best Pract Res Clin Endocrinol Metab 19(2):245–263

    Article  Google Scholar 

  24. Scarsbrook AF, Thakker RV, Wass JA, Gleeson FV, Phillips RR (2006) Multiple endocrine neoplasia: spectrum of radiologic appearances and discussion of a multitechnique imaging approach. Radiographics 26:433–451

    Article  PubMed  Google Scholar 

  25. Maroun J, Kocha W, Kvols L et al (2006) 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 13(2):67–76

    CAS  PubMed  PubMed Central  Google Scholar 

  26. Ramage et al (2012) Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours (NETs). Gut 61:6e32. doi:10.1136/gutjnl-2011-300831

    Article  Google Scholar 

  27. Kaltsas GA, Mukherjee JJ, Grossman AB (2001) The value of radiolabelled MIBG and octreotide in the diagnosis and management of neuroendocrine tumours. Ann Oncol 12(Suppl 2):S47e50

    Article  Google Scholar 

  28. lhan H, Fendler WP, Cyran CC et al (2015) Impact of Ga-DOTATATE PET/CT on the surgical management of primary neuroendocrine tumors of the pancreas or ileum. Ann Surg On- col 22:164–171

    Google Scholar 

  29. Yakemchuk VN, Jager PL, Chirakal R, Reid R, Major P, Gulenchyn KY (2012) PET/CT using 18F-FDOPA provides improved staging of carcinoid tumor patients in a Canadian setting. Nucl Med Commun 33(3):322–330

    Article  CAS  PubMed  Google Scholar 

  30. Koopmans KP J, Neels OC, Kema IP, Elsinga PH, Sluiter WJ, Vanghillewe K, Brouwers AH, Jager PL, de Vries EG (2008) Improved staging of patients with carcinoid and islet cell tumors with 18F-dihydroxy-phenyl-alanine and 11C-5-hy-droxy-tryptophan positron emission tomography. J Clin Oncol 26:1489–1495

    Article  PubMed  Google Scholar 

  31. KlöppelG PA, Heitz PU (2004) The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification. Ann N Y Acad Sci 1014:13–27

    Article  Google Scholar 

  32. Ferrone CR, Tang LH, Tomlinson J et al (2007) Determining prognosis in patients with pancreatic endocrine neoplasms: can the WHO classification system be simplified? J Clin Oncol 25:5609–5615

    Article  PubMed  Google Scholar 

  33. R Srirajaskanthan, A Ahmed, A Prachialias et al (2013) ENETS TNM staging predicts prognosis in small bowel neuroendocrine tumours. ISRN Oncology 420795:7 pages. doi: 10.1155/2013/420795

  34. Jann H, Roll S, Couvelard A et al (2011) Neuroendocrine tumors of midgut and hindgut origin: tumor-node-metastasis classification determines clinical outcome. Cancer 117(15):3332–3341

    Article  PubMed  Google Scholar 

  35. Pape UF et al (2008b) Prognostic relevance of a novel TNM classification system for upper gastroenteropancreatic neuroendocrine tumors. Cancer 113:256–265

    Article  PubMed  Google Scholar 

  36. Strosberg JR, Cheema A, Weber J et al (2011) Prognostic validity of a novel American Joint Committee on Cancer staging classification for pancreatic neuroendocrine tumors. J Clin Oncol 29:3044–3049

    Article  PubMed  Google Scholar 

  37. Rindi G, Falconi M, Klersy C et al (2012) TNM staging of neoplasms of the endocrine pancreas: results from a large international cohort study. J Natl Cancer Inst 104:764–777

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to John K. Ramage .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG

About this chapter

Cite this chapter

Akbar, T., Srirajaskanthan, R., Ramage, J.K. (2018). Tumour Staging: Ileum. 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_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-59024-0_14

  • 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