UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Epidemiology, clinical features, and types of small bowel neoplasms

Authors
James C Cusack, Jr, MD
Michael J Overman, MD
Section Editor
Kenneth K Tanabe, MD
Deputy Editor
Diane MF Savarese, MD

INTRODUCTION

The diagnosis of small bowel tumors is often difficult due to the rarity of these lesions and the nonspecific and variable nature of the presenting signs and symptoms. Thus, delay in diagnosis is common, which may result in the discovery of disease at a late stage and a poor treatment outcome.

Several tumors can arise within the small bowel, both malignant (adenocarcinoma, carcinoid, lymphoma, and sarcomas) and benign (adenoma, leiomyoma, lipoma). Epidemiology, clinical manifestations, and specific tumor types will be reviewed here. Diagnosis, staging, and management of small bowel tumors are discussed separately. (See "Diagnosis and staging of small bowel neoplasms" and "Treatment of small bowel neoplasms".)

TYPES OF TUMORS

A variety of tumors, both malignant and benign, may arise within the small intestine.

Malignant tumors — The distribution of histologic types of small bowel malignant tumors is changing, largely because of the increasing incidence of carcinoids. In 1987, the most common histologic types of malignant tumors of the small intestine in population-based registry data from the Surveillance, Epidemiology and End Results (SEER) program of the National Cancer Institute (NCI) were adenocarcinoma, 45 percent; carcinoid, 29 percent; lymphoma, 16 percent; and sarcoma, 10 percent [1]

In the year 2000, carcinoid tumors surpassed adenocarcinomas as the most common small bowel tumor reported to the National Cancer Data Base (NCDB) [2]. Between 1985 and 2005, the proportion of patients with carcinoids increased from 28 to 44 percent, while the proportion of adenocarcinoma decreased from 42 to 33 percent. The proportion of patients with stromal tumors and lymphoma remained essentially stable (17 and 8 percent, respectively).

                        

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Wed Sep 21 00:00:00 GMT+00:00 2016.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Weiss NS, Yang CP. Incidence of histologic types of cancer of the small intestine. J Natl Cancer Inst 1987; 78:653.
  2. 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; 249:63.
  3. McLaughlin PD, Maher MM. Primary malignant diseases of the small intestine. AJR Am J Roentgenol 2013; 201:W9.
  4. Gabos S, Berkel J, Band P, et al. Small bowel cancer in western Canada. Int J Epidemiol 1993; 22:198.
  5. DiSario JA, Burt RW, Vargas H, McWhorter WP. Small bowel cancer: epidemiological and clinical characteristics from a population-based registry. Am J Gastroenterol 1994; 89:699.
  6. Hatzaras I, Palesty JA, Abir F, et al. Small-bowel tumors: epidemiologic and clinical characteristics of 1260 cases from the connecticut tumor registry. Arch Surg 2007; 142:229.
  7. Lepage C, Bouvier AM, Manfredi S, et al. Incidence and management of primary malignant small bowel cancers: a well-defined French population study. Am J Gastroenterol 2006; 101:2826.
  8. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2016. CA Cancer J Clin 2016; 66:7.
  9. DeSesso JM, Jacobson CF. Anatomical and physiological parameters affecting gastrointestinal absorption in humans and rats. Food Chem Toxicol 2001; 39:209.
  10. Chow WH, Linet MS, McLaughlin JK, et al. Risk factors for small intestine cancer. Cancer Causes Control 1993; 4:163.
  11. Severson RK, Schenk M, Gurney JG, et al. Increasing incidence of adenocarcinomas and carcinoid tumors of the small intestine in adults. Cancer Epidemiol Biomarkers Prev 1996; 5:81.
  12. Haselkorn T, Whittemore AS, Lilienfeld DE. Incidence of small bowel cancer in the United States and worldwide: geographic, temporal, and racial differences. Cancer Causes Control 2005; 16:781.
  13. Neugut AI, Jacobson JS, Suh S, et al. The epidemiology of cancer of the small bowel. Cancer Epidemiol Biomarkers Prev 1998; 7:243.
  14. Chow JS, Chen CC, Ahsan H, Neugut AI. A population-based study of the incidence of malignant small bowel tumours: SEER, 1973-1990. Int J Epidemiol 1996; 25:722.
  15. Scélo G, Boffetta P, Hemminki K, et al. Associations between small intestine cancer and other primary cancers: an international population-based study. Int J Cancer 2006; 118:189.
  16. Zar N, Garmo H, Holmberg L, Hellman P. Risk of second primary malignancies and causes of death in patients with adenocarcinoma and carcinoid of the small intestine. Eur J Cancer 2008; 44:718.
  17. Neugut AI, Santos J. The association between cancers of the small and large bowel. Cancer Epidemiol Biomarkers Prev 1993; 2:551.
  18. Wheeler JM, Warren BF, Mortensen NJ, et al. An insight into the genetic pathway of adenocarcinoma of the small intestine. Gut 2002; 50:218.
  19. Raghav K, Overman MJ. Small bowel adenocarcinomas--existing evidence and evolving paradigms. Nat Rev Clin Oncol 2013; 10:534.
  20. Perzin KH, Bridge MF. Adenomas of the small intestine: a clinicopathologic review of 51 cases and a study of their relationship to carcinoma. Cancer 1981; 48:799.
  21. Rodriguez-Bigas MA, Vasen HF, Lynch HT, et al. Characteristics of small bowel carcinoma in hereditary nonpolyposis colorectal carcinoma. International Collaborative Group on HNPCC. Cancer 1998; 83:240.
  22. Zhang MQ, Chen ZM, Wang HL. Immunohistochemical investigation of tumorigenic pathways in small intestinal adenocarcinoma: a comparison with colorectal adenocarcinoma. Mod Pathol 2006; 19:573.
  23. Giardiello FM, Brensinger JD, Tersmette AC, et al. Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology 2000; 119:1447.
  24. 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; 45:1496.
  25. Kawashima A, Goldman SM, Fishman EK, et al. CT of intraabdominal desmoid tumors: is the tumor different in patients with Gardner's disease? AJR Am J Roentgenol 1994; 162:339.
  26. Canavan C, Abrams KR, Mayberry JF. Meta-analysis: mortality in Crohn's disease. Aliment Pharmacol Ther 2007; 25:861.
  27. Solem CA, Harmsen WS, Zinsmeister AR, Loftus EV Jr. Small intestinal adenocarcinoma in Crohn's disease: a case-control study. Inflamm Bowel Dis 2004; 10:32.
  28. Jess T, Winther KV, Munkholm P, et al. Intestinal and extra-intestinal cancer in Crohn's disease: follow-up of a population-based cohort in Copenhagen County, Denmark. Aliment Pharmacol Ther 2004; 19:287.
  29. Green PH, Jabri B. Celiac disease and other precursors to small-bowel malignancy. Gastroenterol Clin North Am 2002; 31:625.
  30. Howdle PD, Jalal PK, Holmes GK, Houlston RS. Primary small-bowel malignancy in the UK and its association with coeliac disease. QJM 2003; 96:345.
  31. Green PH, Cellier C. Celiac disease. N Engl J Med 2007; 357:1731.
  32. Wu AH, Yu MC, Mack TM. Smoking, alcohol use, dietary factors and risk of small intestinal adenocarcinoma. Int J Cancer 1997; 70:512.
  33. Cross AJ, Leitzmann MF, Subar AF, et al. A prospective study of meat and fat intake in relation to small intestinal cancer. Cancer Res 2008; 68:9274.
  34. Chen CC, Neugut AI, Rotterdam H. Risk factors for adenocarcinomas and malignant carcinoids of the small intestine: preliminary findings. Cancer Epidemiol Biomarkers Prev 1994; 3:205.
  35. Negri E, Bosetti C, La Vecchia C, et al. Risk factors for adenocarcinoma of the small intestine. Int J Cancer 1999; 82:171.
  36. Bjørge T, Tretli S, Engeland A. Height and body mass index in relation to cancer of the small intestine in two million Norwegian men and women. Br J Cancer 2005; 93:807.
  37. Wolk A, Gridley G, Svensson M, et al. A prospective study of obesity and cancer risk (Sweden). Cancer Causes Control 2001; 12:13.
  38. Samanic C, Gridley G, Chow WH, et al. Obesity and cancer risk among white and black United States veterans. Cancer Causes Control 2004; 15:35.
  39. Heymann MF, Hamy A, Triau S, et al. Endocrine tumors of the duodenum. A study of 55 cases relative to clinicopathological features and hormone content. Hepatogastroenterology 2004; 51:1367.
  40. Leoncini E, Carioli G, La Vecchia C, et al. Risk factors for neuroendocrine neoplasms: a systematic review and meta-analysis. Ann Oncol 2016; 27:68.
  41. Ciresi DL, Scholten DJ. The continuing clinical dilemma of primary tumors of the small intestine. Am Surg 1995; 61:698.
  42. Minardi AJ Jr, Zibari GB, Aultman DF, et al. Small-bowel tumors. J Am Coll Surg 1998; 186:664.
  43. Ojha A, Zacherl J, Scheuba C, et al. Primary small bowel malignancies: single-center results of three decades. J Clin Gastroenterol 2000; 30:289.
  44. Talamonti MS, Goetz LH, Rao S, Joehl RJ. Primary cancers of the small bowel: analysis of prognostic factors and results of surgical management. Arch Surg 2002; 137:564.
  45. DARLING RC, WELCH CE. Tumors of the small intestine. N Engl J Med 1959; 260:397.
  46. Garcia Marcilla JA, Sanchez Bueno F, Aguilar J, Parrilla Paricio P. Primary small bowel malignant tumors. Eur J Surg Oncol 1994; 20:630.
  47. Michelassi F, Testa G, Pomidor WJ, et al. Adenocarcinoma complicating Crohn's disease. Dis Colon Rectum 1993; 36:654.
  48. Lynch HT, Smyrk TC, Lynch PM, et al. Adenocarcinoma of the small bowel in lynch syndrome II. Cancer 1989; 64:2178.
  49. MOERTEL CG, SAUER WG, DOCKERTY MB, BAGGENSTOSS AH. Life history of the carcinoid tumor of the small intestine. Cancer 1961; 14:901.
  50. Saha S, Hoda S, Godfrey R, et al. Carcinoid tumors of the gastrointestinal tract: a 44-year experience. South Med J 1989; 82:1501.
  51. Dabaja BS, Suki D, Pro B, et al. Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients. Cancer 2004; 101:518.
  52. Halfdanarson TR, McWilliams RR, Donohue JH, Quevedo JF. A single-institution experience with 491 cases of small bowel adenocarcinoma. Am J Surg 2010; 199:797.
  53. Ross RK, Hartnett NM, Bernstein L, Henderson BE. Epidemiology of adenocarcinomas of the small intestine: is bile a small bowel carcinogen? Br J Cancer 1991; 63:143.
  54. Ribeiro MB, Greenstein AJ, Heimann TM, et al. Adenocarcinoma of the small intestine in Crohn's disease. Surg Gynecol Obstet 1991; 173:343.
  55. Widmar M, Greenstein AJ, Sachar DB, et al. Small bowel adenocarcinoma in Crohn's disease. J Gastrointest Surg 2011; 15:797.
  56. 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; 11:828.
  57. Howe JR, Karnell LH, Menck HR, Scott-Conner C. 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; 86:2693.
  58. AJCC (American Joint Committee on Cancer) Cancer Staging Manual, 7th ed, Edge, SB, Byrd, DR, Compton, CC, et al (Eds), Springer, New York 2010. p.181.
  59. Niederle B, Pape UF, Costa F, et al. ENETS Consensus Guidelines Update for Neuroendocrine Neoplasms of the Jejunum and Ileum. Neuroendocrinology 2016; 103:125.
  60. DAWSON IM, CORNES JS, MORSON BC. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961; 49:80.
  61. Cooper MJ, Williamson RC. Enteric adenoma and adenocarcinoma. World J Surg 1985; 9:914.
  62. Sweetenham JW, Mead GM, Wright DH, et al. Involvement of the ileocaecal region by non-Hodgkin's lymphoma in adults: clinical features and results of treatment. Br J Cancer 1989; 60:366.
  63. 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; 19:3861.
  64. Domizio P, Owen RA, Shepherd NA, et al. Primary lymphoma of the small intestine. A clinicopathological study of 119 cases. Am J Surg Pathol 1993; 17:429.
  65. Turowski GA, Basson MD. Primary malignant lymphoma of the intestine. Am J Surg 1995; 169:433.
  66. Harris NL, Jaffe ES, Stein H, et al. A revised European-American classification of lymphoid neoplasms: a proposal from the International Lymphoma Study Group. Blood 1994; 84:1361.
  67. Harris NL, Jaffe ES, Diebold J, et al. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues: report of the Clinical Advisory Committee meeting-Airlie House, Virginia, November 1997. J Clin Oncol 1999; 17:3835.
  68. Isaacson PG. Gastrointestinal lymphoma. Hum Pathol 1994; 25:1020.
  69. Devaney K, Jaffe ES. The surgical pathology of gastrointestinal Hodgkin's disease. Am J Clin Pathol 1991; 95:794.
  70. Howe JR, Karnell LH, Scott-Conner C. Small bowel sarcoma: analysis of survival from the National Cancer Data Base. Ann Surg Oncol 2001; 8:496.
  71. Shimizu N, Kuramoto S, Mimura T, et al. Leiomyosarcoma originating in Meckel's diverticulum: report of a case and a review of 59 cases in the English literature. Surg Today 1997; 27:546.
  72. 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; 27:625.
  73. Blanchard DK, Budde JM, Hatch GF 3rd, et al. Tumors of the small intestine. World J Surg 2000; 24:421.
  74. FARMER RG, HAWK WA. METASTATIC TUMORS OF THE SMALL BOWEL. Gastroenterology 1964; 47:496.
  75. Kadakia SC, Parker A, Canales L. Metastatic tumors to the upper gastrointestinal tract: endoscopic experience. Am J Gastroenterol 1992; 87:1418.
  76. Richie RE, Reynolds VH, Sawyers JL. Tumor metastases to the small bowel from extra-abdominal sites. South Med J 1973; 66:1383.
  77. Elsayed AM, Albahra M, Nzeako UC, Sobin LH. Malignant melanomas in the small intestine: a study of 103 patients. Am J Gastroenterol 1996; 91:1001.
  78. Lens M, Bataille V, Krivokapic Z. Melanoma of the small intestine. Lancet Oncol 2009; 10:516.
  79. Kemp CD, Russell RT, Sharp KW. Resection of benign duodenal neoplasms. Am Surg 2007; 73:1086.
  80. Witteman BJ, Janssens AR, Griffioen G, Lamers CB. Villous tumours of the duodenum. An analysis of the literature with emphasis on malignant transformation. Neth J Med 1993; 42:5.
  81. Seifert E, Schulte F, Stolte M. Adenoma and carcinoma of the duodenum and papilla of Vater: a clinicopathologic study. Am J Gastroenterol 1992; 87:37.
  82. Spigelman AD, Williams CB, Talbot IC, et al. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet 1989; 2:783.
  83. Standards of Practice Committee, Adler DG, Qureshi W, et al. The role of endoscopy in ampullary and duodenal adenomas. Gastrointest Endosc 2006; 64:849.
  84. Apel D, Jakobs R, Weickert U, Riemann JF. High frequency of colorectal adenoma in patients with duodenal adenoma but without familial adenomatous polyposis. Gastrointest Endosc 2004; 60:397.
  85. Schneider AR, Seifert H, Trojan J, et al. Frequency of colorectal polyps in patients with sporadic adenomas or adenocarcinomas of the papilla of vater--an age-matched, controlled study. Z Gastroenterol 2005; 43:1123.
  86. Murray MA, Zimmerman MJ, Ee HC. Sporadic duodenal adenoma is associated with colorectal neoplasia. Gut 2004; 53:261.
  87. Dudiak KM, Johnson CD, Stephens DH. Primary tumors of the small intestine: CT evaluation. AJR Am J Roentgenol 1989; 152:995.