Skip to main content
SpringerLink
Log in

Small Bowel Neoplasms and Polyps

  • Small Intestine (D Sachar, Section Editor)
  • Published:
Current Gastroenterology Reports Aims and scope Submit manuscript

Abstract

The small intestine is a relatively privileged organ that only rarely develops malignant or even benign tumors. Given this rarity, the relative inaccessibility of the organ during routine endoscopic procedures, and the typical absence or nonspecific nature of clinical manifestations, these tumors often go undiagnosed. Treatment and prognosis are tailored to each histological subtype of tumor. This chapter will discuss the epidemiology, presentation, diagnostics, and management for the most common small bowel tumors, and will highlight the importance of recognizing patients at higher risk of small bowel neoplasia.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2015. CA Cancer J Clin. 2015;65(1):5.

    Article  PubMed  Google Scholar 

  2. Weiss NS, Yang CP. Incidence of histologic types of cancer of the small intestine. J Natl Cancer Inst. 1987;78(4):653.

    CAS  PubMed  Google Scholar 

  3. Bilimoria KY, Bentrem DJ, Wayne JD, et al. Small bowel cancer in the United States: changes in epidemiology, treatment, and survival over the last 20 years. Ann Surg. 2009;249(1):63.

    Article  PubMed  Google Scholar 

  4. Aparicio T, Zaanan A, Svrcek M, et al. Small bowel adenocarcinoma: epidemiology, risk factors, diagnosis and treatment. Dig Liver Dis. 2014;46:97–104.

    Article  PubMed  Google Scholar 

  5. Laurent F, Raynaud M, Biset JM, et al. Diagnosis and categorization of small bowel neoplasms: role of computed tomography. Gastrointest Radiol. 1991;16(2):115.

    Article  CAS  PubMed  Google Scholar 

  6. Pilleul F, Penigaud M, Milot L, et al. Possible small-bowel neoplasms: contrast-enhanced and water-enhanced multidetector CT enteroclysis. Radiology. 2006;241(3):796.

    Article  PubMed  Google Scholar 

  7. Pappalardo G, Gualdi G, Nunziale A, et al. Impact of magnetic resonance in the preoperative staging and the surgical planning for treating small bowel neoplasms. Surg Today. 2013;43(6):613–9.

    Article  PubMed  Google Scholar 

  8. Kunz PL, Reidy-Lagunes D, Anthony LB, et al. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013;42(4):557–77.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Pape UF, Perren A, Niederle B, et al. ENETS consensus guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology. 2012;95:135–56.

    Article  CAS  PubMed  Google Scholar 

  10. Sainz-Esteban A, Olmos R, Gonzalez-Sagrado M, et al. Contribution of 111In-pentetreotide SPECT/CT imaging to conventional somatostatin receptor scintigraphy in the detection of neuroendocrine tumours. Nucl Med Commun. 2015;36:251–9.

    Article  CAS  PubMed  Google Scholar 

  11. Lewis BS, Eisen GM, Friedman S. A pooled analysis to evaluate results of capsule endoscopy trials. Endoscopy. 2005;37(10):960.

    Article  CAS  PubMed  Google Scholar 

  12. Haanstra J, Al-Toma A, Dekker E, et al. Prevalence of small-bowel neoplasia in Lynch syndrome assessed by video capsule endoscopy. Gut. 2015;64:1578–83. Large, prospective study to assess if video capsule endoscopy was a useful screening tool for small bowel tumors in patients with Lynch syndrome. The results showed that the three patients who developed small bowel tumors had them all within reach of a gastroduodenoscope in the duodenum, one of which was missed on a capsule study, suggesting that capsule endoscopy should be used not universally but in specific clinical scenarios.

    Article  PubMed  Google Scholar 

  13. Berner JS, Mauer K, Lewis BS. Push and sonde enteroscopy for the diagnosis of obscure gastrointestinal bleeding. Am J Gastroenterol. 1994;89:2139–42.

    CAS  PubMed  Google Scholar 

  14. Triester SL, Leighton JA, Leontiadis GI, et al. A meta-analysis of the yield of capsule endoscopy compared to other diagnostic modalities in patients with obscure gastrointestinal bleeding. Am J Gastroenterol. 2005;100:2407–18.

    Article  PubMed  Google Scholar 

  15. Islam S, Leighton JA, Pasha SF. Evaluation and management of small-bowel tumors in the era of deep enteroscopy. Gastrointest Endosc. 2014;79:732–40.

    Article  PubMed  Google Scholar 

  16. Chen W-G, Shan G-D, Zhang H, et al. Double-balloon enteroscopy in small bowel tumors: a Chinese single-center study. World J Gastroenterol : WJG. 2013;19(23):3665–71.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Pasha SF, Leighton JA, Das A, et al. Double-balloon enteroscopy and capsule endoscopy have comparable diagnostic yield in small-bowel disease: a meta-analysis. Clin Gastroenterol Hepatol. 2008;6:671–6.

    Article  PubMed  Google Scholar 

  18. Talamonti MS, Goetz LH, Rao S, et al. Primary cancers of the small bowel: analysis of prognostic factors and results of surgical management. Arch Surg. 2002;137(5):564.

    Article  PubMed  Google Scholar 

  19. Bajetta E, Ferrari L, Martinetti A, et al. Chromogranin A, neuron specific enolase, carcinoembryonic antigen, and hydroxyindole acetic acid evaluation in patients with neuroendocrine tumors. Cancer. 1999;86:858–65.

    Article  CAS  PubMed  Google Scholar 

  20. Raghav K, Overman MJ. Small bowel adenocarcinomas—existing evidence and evolving paradigms. Nat Rev Clin Oncol. 2013;10(9):534–44.

    Article  CAS  PubMed  Google Scholar 

  21. Halfdanarson TR, McWilliams RR, Donohue JH, et al. A single-institution experience with 491 cases of small bowel adenocarcinoma. Am J Surg. 2010;199(6):797.

    Article  PubMed  Google Scholar 

  22. Aparicio T, Svrcek M, Zaanan A, et al. Small bowel adenocarcinoma phenotyping, a clinicobiological prognostic study. Br J Cancer. 2013;109:3057–66. One of the largest studies assessing the clinical and biological characteristics of small bowel adenocarcinoma in 63 patients that showed a similarity between the pathogenesis of small bowel adenocarcinoma and colorectal carcinoma, and less similarity with gastric adenocarcinoma.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. T Howe JR, Karnell LH, Menck HR, et al. The American College of Surgeons Commission on Cancer and the American Cancer Society. Adenocarcinoma of the small bowel: review of the National Cancer Data Base, 1985–1995. Cancer. 1999;86(12):2693.

    Article  Google Scholar 

  24. Dabaja BS, Suki D, Pro B, et al. Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 2 patients. Cancer. 2004;101(3):518.

    Article  PubMed  Google Scholar 

  25. Sohn TA, Lillemoe KD, Cameron JL, et al. Adenocarcinoma of the duodenum: factors influencing long-term survival. J Gastrointest Surg. 1998;2(1):79.

    Article  CAS  PubMed  Google Scholar 

  26. Abrahams NA, Halverson A, Fazio VW, et al. Adenocarcinoma of the small bowel: a study of 37 cases with emphasis on histologic prognostic factors. Dis Colon Rectum. 2002;45(11):1496.

    Article  PubMed  Google Scholar 

  27. Ecker BL, McMillan MT, Datta J, et al. Efficacy of adjuvant chemotherapy for small bowel adenocarcinoma: a propensity score-matched analysis. Cancer. 2015. doi:10.1002/cncr.29840. This study shows that adjuvant chemotherapy provided higher median survival for stage III adenocarcinoma patients as compared to a group that received surgery alone.

    PubMed  Google Scholar 

  28. Syngal S, Brand RE, Church JM, et al. ACG Clinical Guideline: genetic testing and management of hereditary gastrointestinal cancer syndromes. Am J Gastroenterol. 2015;110:223–63.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Koornstra JJ. Small bowel endoscopy in familial adenomatous polyposis and Lynch syndrome. Best Pract Res Clin Gastroenterol. 2012;26:359–68.

    Article  PubMed  Google Scholar 

  30. Giardiello FM, Brensinger JD, Tersmette AC, et al. Very high risk of cancer in familial Peutz–Jeghers syndrome. Gastroenterology. 2000;119:1447–53.

    Article  CAS  PubMed  Google Scholar 

  31. Win AK, Lindor NM, Young JP, et al. Risks of primary extracolonic cancers following colorectal cancer in lynch syndrome. J Natl Cancer Inst. 2012;104:1363–72.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Koornstra JJ, Kleibeuker JH, Vasen HF. Small-bowel cancer in Lynch syndrome: is it time for surveillance? Lancet Oncol. 2008;9:901–05.

    Article  PubMed  Google Scholar 

  33. Beaugerie L, Itzkowitz SH. Cancers complicating inflammatory bowel disease. N Engl J Med. 2015;372:1441–52.

    Article  CAS  PubMed  Google Scholar 

  34. Palascak-Juif V, Bouvier AM, Cosnes J, et al. Small bowel adenocarcinoma in patients with Crohn’s disease compared with small bowel adenocarcinoma de novo. Inflamm Bowel Dis. 2005;11(9):828.

    Article  PubMed  Google Scholar 

  35. Cahill C, Gordon PH, Petrucci A, et al. Small bowel adenocarcinoma and Crohn’s disease: any further ahead than 50 years ago? WJG. 2014;20(33):11486–95.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Michelassi F, Testa G, Pomidor WJ, et al. Adenocarcinoma complicating Crohn’s disease. Dis Colon Rectum. 1993;36(7):654.

    Article  CAS  PubMed  Google Scholar 

  37. Swinson CM, Slavin G, Coles EC, et al. Coeliac disease and malignancy. Lancet. 1983;1:111–5.

    Article  CAS  PubMed  Google Scholar 

  38. Askling J, Linet M, Gridley G, et al. Cancer incidence in a population-based cohort of individuals hospitalized with celiac disease or dermatitis herpetiformis. Gastroenterology. 2002;123:1428–35.

    Article  PubMed  Google Scholar 

  39. Weber HC, Venzon DJ, Lin JT, et al. Determinants of metastatic rate and survival in patients with Zollinger-Ellison syndrome: a prospective long-term study. Gastroenterology. 1995;108(6):1637.

    Article  CAS  PubMed  Google Scholar 

  40. Saha S, Hoda S, Godfrey R, et al. Carcinoid tumors of the gastrointestinal tract: a 44-year experience. South Med J. 1989;82(12):1501.

    Article  CAS  PubMed  Google Scholar 

  41. Kim MK, Warner RR, Roayaie S, et al. Revised staging classification improves outcome prediction for small intestinal neuroendocrine tumors. J Clin Oncol. 2013;31(30):3776–81.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Nikou GC, Lygidakis NJ, Toubanakis C, et al. Current diagnosis and treatment of gastrointestinal carcinoids in a series of 101 patients: the significance of serum chromogranin-A, somatostatin receptor scintigraphy and somatostatin analogues. Hepatogastroenterology. 2005;52(63):731.

    PubMed  Google Scholar 

  43. Mayo SC, de Jong MC, Pulitano C, et al. Surgical management of hepatic neuroendocrine tumor metastasis: results from an international multi-institutional analysis. Ann Surg Oncol. 2010;17(12):3129.

    Article  PubMed  Google Scholar 

  44. Sarmiento JM, Heywood G, Rubin J, et al. Surgical treatment of neuroendocrine metastases to the liver: a plea for resection to increase survival. J Am Coll Surg. 2003;197(1):29–37.

    Article  PubMed  Google Scholar 

  45. Koch P, del Valle F, Berdel WE, et al. Primary gastrointestinal non-Hodgkin’s lymphoma: I. Anatomic and histologic distribution, clinical features, and survival data of 371 patients registered in the German Multicenter Study GIT NHL 01/92. J Clin Oncol. 2001;19(18):3861.

    CAS  PubMed  Google Scholar 

  46. Isaacson PG. Gastrointestinal lymphoma. Hum Pathol. 1994;25(10):1020.

    Article  CAS  PubMed  Google Scholar 

  47. Nijeboer P, Malamut G, Mulder CJ, et al. Enteropathy-associated T-cell lymphoma: improving treatment strategies. Dig Dis. 2015;33:231–5.

    Article  CAS  PubMed  Google Scholar 

  48. Miettinen M, Kopczynski J, Makhlouf HR, et al. Gastrointestinal stromal tumors, intramural leiomyomas, and leiomyosarcomas in the duodenum: a clinicopathologic, immunohistochemical, and molecular genetic study of 167 cases. Am J Surg Pathol. 2003;27(5):625.

    Article  PubMed  Google Scholar 

  49. Rubin BP, Fletcher JA, Fletcher CD. Molecular insights into the histogenesis and pathogenesis of gastrointestinal stromal tumors. Int J Surg Pathol. 2000;8(1):5.

    Article  CAS  PubMed  Google Scholar 

  50. Bresalier RS, Blechacz B. Chapter 125. Tumors of the small intestine. In: Felman M, Friedman LS, Brandt LJ, editors. Sleisenger and Fordtran’s gastrointestinal and liver disease, 10th Ed, Saunders Elsevier, 2016. pp. 2196-2212.

  51. Corless CL, Ballman KV, Antonescu CR, et al. Pathologic and molecular features correlate with long-term outcome after adjuvant therapy of resected primary GI stromal tumor: the ACOSOG Z9001 trial. J Clin Oncol. 2014;32(15):1563.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  52. Demetri GD, von Mehren M, Blanke CD, et al. Efficacy and safety of imatinib mesylate in advanced gastrointestinal stromal tumors. N Engl J Med. 2002;347(7):472.

    Article  CAS  PubMed  Google Scholar 

  53. Kemp CD, Russell RT, Sharp KW. Resection of benign duodenal neoplasms. Am Surg. 2007;73(11):1086.

    PubMed  Google Scholar 

  54. Spigelman AD, Williams CB, Talbot IC, et al. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet. 1989;2(8666):783.

    Article  CAS  PubMed  Google Scholar 

  55. Adler DG, Qureshi W, Davila R, et al. The role of endoscopy in ampullary and duodenal adenomas. Gastrointest Endosc. 2006;64(6):849.

    Article  PubMed  Google Scholar 

  56. Murray MA, Zimmerman MJ, Ee HC. Sporadic duodenal adenoma is associated with colorectal neoplasia. Gut. 2004;53(2):261.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. The Dr. Henry D. Janowitz Division of Gastroenterology, Icahn School of Medicine at Mount Sinai, One Gustave Levy Place, GI Division, Box 1069, New York City, NY, 10029, USA

    Kamron Pourmand & Steven H. Itzkowitz

Authors
  1. Kamron Pourmand
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Steven H. Itzkowitz
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Steven H. Itzkowitz.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

Additional information

This article is part of the Topical Collection on Small Intestine

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Pourmand, K., Itzkowitz, S.H. Small Bowel Neoplasms and Polyps. Curr Gastroenterol Rep 18, 23 (2016). https://doi.org/10.1007/s11894-016-0497-x

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11894-016-0497-x

Keywords

Search

Navigation