Small-Bowel Tumors, Polyps, and Polyposis Syndromes

  • Alessandra Bizzotto
  • Maria Elena RiccioniEmail author
  • Rosario Landi
  • Clelia Marmo
  • Brunella Barbaro
  • Guido Costamagna


Despite the fact that the small bowel accounts for the majority of the length of the alimentary tract, small-bowel tumors (SBTs) are rare entities and pose a unique diagnostic and management challenge. Until the advent of capsule endoscopy (CE) and device-assisted enteroscopy (DAE) coupled with the advances in radiology, physicians had limited diagnostic tests for investigating the small bowel. CE allows a direct entire evaluation of the small bowel and has become the frontline diagnostic tool in case of suspected small-bowel diseases. DAE allows histopathological diagnosis and deeper enteroscopy. CT enteroclysis/CT enterography (CTE) provides not only pictures of the luminal side but also information about adjacent organs. Magnetic resonance enteroclysis/enterography compared to CTE offers the advantage of soft tissue contrast and multiplanar imaging without radiation exposure.

Polyps of the small bowel can be inflammatory, hyperplastic, hamartomatous, and adenomas. Hamartomatous polyps and adenomas may arise sporadically or belong to polyposis syndromes that are associated with an increased risk of gastrointestinal cancer. Familial Adenomatous Polyposis (FAP) and Peutz-Jeghers syndrome (PJS) are the most common inherited intestinal polyposis syndromes. The clinical usefulness and cost-effectiveness of routine small-bowel screening and surveillance in FAP patients are not known at this time. The current evidence does not support routine prophylactic examination of the deep small bowel in this disorder. On the other hand, PJS is conversely a domain where CE and DAE best exhibit their diagnostic and therapeutic power.


Small-bowel tumors Small-bowel polyps Polyposis syndromes Capsule endoscopy Device-assisted enteroscopy CT enterography MR enterography Familial adenomatous polyposis Peutz-Jeghers syndrome 

Supplementary material

Video 13.1

Endoscopic biopsy of a large jejunal melanoma (MP4 2,425 kb)

Video 13.2

Very large jejunal polyp on DAE in a patient referred for OGIB (MP4 11,018 kb)

Video 13.3

Demonstration of endoscopic marking of SBT (MP4 9,481 kb)

Video 13.4

DAE with multiple endoscopic polypectomies in PJS (MP4 5,774 kb)


  1. 1.
    Ubaiah O, Devesa SS, Platz CE, et al. Small intestinal cancer: a population-based study of incidence and survival patterns in the United States, 1992 to 2006. Cancer Epidemiol Biomarkers Prev. 2010;19(8):1908–18.Google Scholar
  2. 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(1):63–71.PubMedGoogle Scholar
  3. 3.
    Pan SY, Morrison H. Epidemiology of cancer of the small intestine. World J Gastrointest Oncol. 2011;3(3):33–42. 15.PubMedCentralPubMedGoogle Scholar
  4. 4.
    Neugut AI, Jacobson JS, Suh S, et al. The epidemiology of cancer of the small bowel. Cancer Epidemiol Biomarkers Prev. 1998;7(3):243–51.PubMedGoogle Scholar
  5. 5.
    Raghav K, Overman MJ. Small bowel adenocarcinomas – existing evidence and evolving paradigms. Nat Rev Clin Oncol. 2009;10(9):534–44.Google Scholar
  6. 6.
    Cheung DY, Choi MG. Current advance in small bowel tumors. Clin Endosc. 2011;44(1):13–21.PubMedCentralPubMedGoogle Scholar
  7. 7.
    Dabaja BS, Suki D, Pro B, et al. Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients. Cancer. 2004;101(3):518–26.PubMedGoogle Scholar
  8. 8.
    Farhat MH, Shamseddine AI, Barada KA. Small bowel tumors: clinical presentation, prognosis, and outcome in 33 patients in a tertiary care center. J Oncol. 2008. doi:10.1155/2008/212067. Epub 2008 Dec 2.PubMedCentralPubMedGoogle Scholar
  9. 9.
    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(5):564–70. discussion 570-1.PubMedGoogle Scholar
  10. 10.
    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(5):699–701.PubMedGoogle Scholar
  11. 11.
    Naef M, Bühlmann M, Baer HU. Small bowel tumors: diagnosis, therapy and prognostic factors. Langenbecks Arch Surg. 1999;384(2):176–80.PubMedGoogle Scholar
  12. 12.
    Aparicio T, Zaanan A, Svrcek M, et al. Small bowel adenocarcinoma: epidemiology, risk factors, diagnosis and treatment. Dig Liver Dis. 2014;46(2):97–104.PubMedGoogle Scholar
  13. 13.
    Cananzi FC, Lorenzi B, Belgaumkar A, et al. Prognostic factors for primary gastrointestinal stromal tumors: are they the same in the multidisciplinary treatment era? Langenbecks Arch Surg. 2014;399(3):323–32.PubMedGoogle Scholar
  14. 14.
    Tabrizian P, Sweeney RE, Uhr JH, et al. Laparoscopic resection of gastric and small bowel gastrointestinal stromal tumors: 10-year experience at a single center. J Am Coll Surg. 2014;218(3):367–73.PubMedGoogle Scholar
  15. 15.
    Zuckerman GR, Prakash C, Askin MP, Lewis BS. AGA technical review on the evaluation and management of occult and obscure gastrointestinal bleeding. Gastroenterology. 2000;118(1):201–21.PubMedGoogle Scholar
  16. 16.
    Ekberg O, Ekholm S. Radiography in primary tumors of the small bowel. Acta Radiol Diagn (Stockh). 1980;21(1):79–84.Google Scholar
  17. 17.
    Zollinger Jr RM. Primary neoplasms of the small intestine. Am J Surg. 1986;151(6):654–8.PubMedGoogle Scholar
  18. 18.
    Orjollet-Lecoanet C, Ménard Y, Martins A, et al. CT enteroclysis for detection of small bowel tumors. J Radiol. 2000;81(6):618–27.PubMedGoogle Scholar
  19. 19.
    Bessette JR, Maglinte DD, Kelvin FM, Chernish SM. Primary malignant tumors in the small bowel: a comparison of the small-bowel enema and conventional follow-through examination. AJR Am J Roentgenol. 1989;153(4):741–4.PubMedGoogle Scholar
  20. 20.
    Thoeni RF, Gould RG. Enteroclysis and small bowel series: comaprison of radiation dose and examination time. Radiology. 1991;178:659–62.PubMedGoogle Scholar
  21. 21.
    Gill SS, Heuman DM, Mihas AA. Small intestinal neoplasms. J Clin Gastroenterol. 2001;33:267–82.PubMedGoogle Scholar
  22. 22.
    Soyer P, Boudiaf M, Fishman EK, et al. Imaging of malignant neoplasms of the mesenteric small bowel: new trends and perspectives. Crit Rev Oncol Hematol. 2011;80(1):10–30.PubMedGoogle Scholar
  23. 23.
    Hoeffel C, Mule S, Romaniuk B, et al. Advances in radiological imaging of gastrointestinal tumors. Crit Rev Oncol Hematol. 2009;69(2):153–67.PubMedGoogle Scholar
  24. 24.
    May A, Nachbar L, Schneider M, Ell C. Prospective comparison of push enteroscopy and push-and-pull enteroscopy in patients with suspected small-bowel bleeding. Am J Gastroenterol. 2006;101(9):2016–24.PubMedGoogle Scholar
  25. 25.
    Marmo R, Rotondano G, Piscopo R, et al. Meta-analysis: capsule enteroscopy vs. conventional modalities in diagnosis of small bowel diseases. Aliment Pharmacol Ther. 2005;22(7):595–604.PubMedGoogle Scholar
  26. 26.
    Berner JS, Mauer K, Lewis BS. Push and sonde enteroscopy for the diagnosis of obscure gastrointestinal bleeding. Am J Gastroenterol. 1994;89(12):2139–42.PubMedGoogle Scholar
  27. 27.
    Iacopini G, Frontespezi S, Vitale MA, et al. Routine ileoscopy at colonoscopy: a prospective evaluation of learning curve and skill-keeping line. Gastrointest Endosc. 2006;63(2):250–6.PubMedGoogle Scholar
  28. 28.
    Cheung DY, Lee IS, Chang DK, et al. for the Korean Gut Images Study Group. Capsule endoscopy in small bowel tumors: a multicenter Korean study. J Gastroenterol Hepatol. 2010;25(6):1079–86.Google Scholar
  29. 29.
    Bailey AA, Debinski HS, Appleyard MN, et al. Diagnosis and outcome of small bowel tumors found by capsule endoscopy: a three-center Australian experience. Am J Gastroenterol. 2006;101(10):2237–43.PubMedGoogle Scholar
  30. 30.
    Maieron A, Hubner D, Blaha B, et al. Multicenter retrospective evaluation of capsule endoscopy in clinical routine. Endoscopy. 2004;36(10):864–8.PubMedGoogle Scholar
  31. 31.
    Schulmann K, Hollerbach S, Kraus K, et al. Feasibility and diagnostic utility of video capsule endoscopy for the detection of small bowel polyps in patients with hereditary polyposis syndromes. Am J Gastroenterol. 2005;100(1):27–37.PubMedGoogle Scholar
  32. 32.
    Iaquinto G, Fornasarig M, Quaia M, et al. Capsule endoscopy is useful and safe for small-bowel surveillance in familial adenomatous polyposis. Gastrointest Endosc. 2008;67(1):61–7.PubMedGoogle Scholar
  33. 33.
    Schwartz GD, Barkin JS. Small-bowel tumors detected by wireless capsule endoscopy. Dig Dis Sci. 2007;52:1026–30.PubMedGoogle Scholar
  34. 34.
    de Franchis R, Rondonotti E, Abbiati C, et al. Small bowel malignancy. Gastrointest Endosc Clin N Am. 2004;14(1):139–48.PubMedGoogle Scholar
  35. 35.
    Cobrin GM, Pittman RH, Lewis BS. Increased diagnostic yield of small bowel tumors with capsule endoscopy. Cancer. 2006;107(1):22–7.PubMedGoogle Scholar
  36. 36.
    Estevez E, Gonzalez-Conde B, Vazquez-Iglesias JL, et al. Incidence of tumoral pathology according to study using capsule endoscopy for patients with obscure gastrointestinal bleeding. Surg Endosc. 2007;21:1776–80.PubMedGoogle Scholar
  37. 37.
    Urbain D, De Looze D, Demedts I, et al. Video capsule endoscopy in small-bowel malignancy: a multicenter Belgian study. Endoscopy. 2006;38(4):408–11.PubMedGoogle Scholar
  38. 38.
    Urgesi R, Riccioni ME, Bizzotto A, et al. Increased diagnostic yield of small bowel tumors with PillCam: the role of capsule endoscopy in the diagnosis and treatment of gastrointestinal stromal tumors (GISTs). Italian single-center experience. Tumori. 2012;98(3):357–63.PubMedGoogle Scholar
  39. 39.
    Achour J, Serraj I, Amrani L, Amrani N. Small bowel tumors: what is the contribution of video capsule endoscopy? Clin Res Hepatol Gastroenterol. 2012;36(3):222–6.PubMedGoogle Scholar
  40. 40.
    Rondonotti E, Pennazio M, Toth E, on behalf of the European Capsule Endoscopy Group, the Italian Club for Capsule Endoscopy (CICE) and the Iberian Group for Capsule Endoscopy, et al. Small-bowel neoplasms in patients undergoing video capsule endoscopy: a multicenter European study. Endoscopy. 2008;40(6):488–95.PubMedGoogle Scholar
  41. 41.
    Pasha SF, Sharma VK, Carey EJ, et al. Utility of video capsule endoscopy in the detection of small bowel tumors – a single center experience of 1000 consecutive patients. Presented at ICCE, Madrid, Spain, 8–9 June 2007. Abstract 45. 2007.Google Scholar
  42. 42.
    Leighton JA. The role of endoscopic imaging of the small bowel in clinical practice. Am J Gastroenterol. 2011;106(1):27–36.PubMedGoogle Scholar
  43. 43.
    Spada C, Riccioni ME, Familiari P, et al. Video capsule endoscopy in small-bowel tumors: a single centre experience. Scand J Gastroenterol. 2008;43(4):497–505.PubMedGoogle Scholar
  44. 44.
    Delvaux M, Friedman S, Keuchel M, et al. Structured terminology for capsule endoscopy: results of retrospective testing and validation in 766 small-bowel investigations. Endoscopy. 2005;37(10):945–50.PubMedGoogle Scholar
  45. 45.
    Korman LY, Delvaux M, Gay G, et al. Capsule endoscopy structured terminology (CEST): proposal of a standardized and structured terminology for reporting capsule endoscopy procedures. Endoscopy. 2005;37(10):951–9.PubMedGoogle Scholar
  46. 46.
    Pennazio M, Rondonotti E, de Franchis R. Capsule endoscopy in neoplastic diseases. World J Gastroenterol. 2008;14(34):5245–53.PubMedCentralPubMedGoogle Scholar
  47. 47.
    Pohl J, Delvaux M, Ell C, the ESGE Clinical Guidelines Committee, et al. European Society of Gastrointestinal Endoscopy (ESGE) guidelines: flexible enteroscopy for diagnosis and treatment of small-bowel diseases. Endoscopy. 2008;40(7):609–18.PubMedGoogle Scholar
  48. 48.
    Girelli CM, Porta P, Colombo E, et al. Development of a novel index to discriminate bulge from mass on small-bowel capsule endoscopy. Gastrointest Endosc. 2011;74(5):1067–74.PubMedGoogle Scholar
  49. 49.
    Barbosa DC, Roupar DB, Ramos JC, et al. Automatic small bowel tumor diagnosis by using multi-scale wavelet-based analysis in wireless capsule endoscopy images. Biomed Eng Online. 2012;11:3.PubMedCentralPubMedGoogle Scholar
  50. 50.
    Pasha SF, Hara AK, Leighton JA. Diagnostic evaluation and management of obscure gastrointestinal bleeding: a changing paradigm. Gastroenterol Hepatol (NY). 2009;5(12):839–50.Google Scholar
  51. 51.
    Zagorowicz ES, Pietrzak AM, Wronska E, et al. Small bowel tumors detected and missed during capsule endoscopy: single center experience. World J Gastroenterol. 2013;19(47):9043–8.PubMedCentralPubMedGoogle Scholar
  52. 52.
    Flieger D, Keller R, May A, et al. Capsule endoscopy in gastrointestinal lymphomas. Endoscopy. 2005;37:1174–80.PubMedGoogle Scholar
  53. 53.
    Daum S, Wahnschaffe U, Glasenapp R, et al. Capsule endoscopy in refractory celiac disease. Endoscopy. 2007;39:455–8.PubMedGoogle Scholar
  54. 54.
    Baichi MM, Arifuddin RM, Mantry PS. Small-bowel masses found and missed on capsule endoscopy for obscure bleeding. Scand J Gastroenterol. 2007;42(9):1127–32.PubMedGoogle Scholar
  55. 55.
    Lewis BS, Eisen GM, Friedman S. A pooled analysis to evaluate results of capsule endoscopy trials. Endoscopy. 2005;37:960–5.PubMedGoogle Scholar
  56. 56.
    Hakim FA, Alexander JA, Huprich JE, et al. CT-enterography may identify small bowel tumors not detected by capsule endoscopy: eight years experience at Mayo Clinic Rochester. Dig Dis Sci. 2011;56(10):2914–9.PubMedGoogle Scholar
  57. 57.
    Ohmiya N, Nakamura M, Tahara T, et al. Management of small-bowel polyps at double-balloon enteroscopy. Ann Transl Med. 2014;2(3):30.PubMedCentralPubMedGoogle Scholar
  58. 58.
    Ross A, Mehdizadeh S, Tokar J, et al. Double balloon enteroscopy detects small bowel mass lesions missed by capsule endoscopy. Dig Dis Sci. 2008;53(8):2140–3.PubMedGoogle Scholar
  59. 59.
    Honda W, Ohmiya N, Hirooka Y, et al. Enteroscopic and radiologic diagnoses, treatment, and prognoses of small-bowel tumors. Gastrointest Endosc. 2012;76(2):344–54.PubMedGoogle Scholar
  60. 60.
    Liao Z, Gao R, Xu C, Li ZS. Indications and detection, completion, and retention rates of small-bowel capsule endoscopy: a systematic review. Gastrointest Endosc. 2010;71(2):280–6.PubMedGoogle Scholar
  61. 61.
    Riccioni ME, Urgesi R, Cianci R, et al. Current status of device-assisted enteroscopy: technical matters, indication, limits and complications. World J Gastrointest Endosc. 2012;4(10):453–61.PubMedCentralGoogle Scholar
  62. 62.
    Tennyson CA, Lewis BS. Enteroscopy: an overview. Gastrointest Endosc Clin N Am. 2009;19(3):315–24.PubMedGoogle Scholar
  63. 63.
    Islam RS, Leighton JA, Pasha SF. Evaluation and management of small-bowel tumors in the era of deep enteroscopy. Gastrointest Endosc. 2014;79(5):732–40.PubMedGoogle Scholar
  64. 64.
    Mensink PB, Haringsma J, Kucharzik T, et al. Complications of double balloon enteroscopy: a multicenter survey. Endoscopy. 2007;39(7):613–5.PubMedGoogle Scholar
  65. 65.
    Aktas H, de Ridder L, Haringsma J, et al. Complications of single-balloon enteroscopy: a prospective evaluation of 166 procedures. Endoscopy. 2010;42(5):365–8.PubMedGoogle Scholar
  66. 66.
    Partridge BJ, Tokar JL, Haluszka O, Heller SJ. Small bowel cancers diagnosed by device-assisted enteroscopy at a U.S. referral center: a five-year experience. Dig Dis Sci. 2011;56(9):2701–5.PubMedGoogle Scholar
  67. 67.
    Lee BI, Choi H, Choi KY, et al. Clinical characteristics of small bowel tumors diagnosed by double-balloon endoscopy: KASID multi-center study. Dig Dis Sci. 2011;56(10):2920–7.PubMedGoogle Scholar
  68. 68.
    Mitsui K, Tanaka S, Yamamoto H, et al. Role of double-balloon endoscopy in the diagnosis of small-bowel tumors: the first Japanese multicenter study. Gastrointest Endosc. 2009;70(3):498–504.PubMedGoogle Scholar
  69. 69.
    Cangemi DJ, Patel MK, Gomez V, et al. Small bowel tumors discovered during double-balloon enteroscopy: analysis of a large prospectively collected single-center database. J Clin Gastroenterol. 2013;47(9):769–72.PubMedGoogle Scholar
  70. 70.
    Chen W, Shan GD, Zhang H, et al. Double-balloon enteroscopy in small bowel tumors: a Chinese single-center study. World J Gastroenterol. 2013;19(23):3665–71.PubMedCentralPubMedGoogle Scholar
  71. 71.
    Fry LC, Neumann H, Kuester D, et al. Small bowel polyps and tumors: endoscopic detection and treatment by double-balloon enteroscopy. Aliment Pharmacol Ther. 2009;29(1):135–42.PubMedGoogle Scholar
  72. 72.
    Bellutti M, Fry LC, Schmitt J, et al. Detection of neuroendocrine tumors of the small bowel by double balloon enteroscopy. Dig Dis Sci. 2009;54(5):1050–8.PubMedGoogle Scholar
  73. 73.
    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(6):671–6.PubMedGoogle Scholar
  74. 74.
    Hadithi M, Heine GD, Jacobs MA, et al. A prospective study comparing video capsule endoscopy with double-balloon enteroscopy in patients with obscure gastrointestinal bleeding. Am J Gastroenterol. 2006;101:52–7.PubMedGoogle Scholar
  75. 75.
    Nakamura T, Terano A. Capsule endoscopy: past, present, and future. J Gastroenterol. 2008;43:93–9.PubMedGoogle Scholar
  76. 76.
    Gay G, Delvaux M, Fassler I. Outcome of capsule endoscopy in determining indication and route for push-and-pull enteroscopy. Endoscopy. 2006;38(1):49–58.PubMedGoogle Scholar
  77. 77.
    Kaffes AJ, Siah C, Koo JH. Clinical outcomes after double-balloon enteroscopy in patients with obscure GI bleeding and a positive capsule endoscopy. Gastrointest Endosc. 2007;66:304–9.PubMedGoogle Scholar
  78. 78.
    Hendel JW, Vilmann P, Jensen T. Double-balloon endoscopy: who needs it? Scand J Gastroenterol. 2008;43:363–7.PubMedGoogle Scholar
  79. 79.
    Jones BH, Fleischer DE, Sharma VK, et al. Yield of repeat wireless video capsule endoscopy in patients with obscure gastrointestinal bleeding. Am J Gastroenterol. 2005;100:1058–64.PubMedGoogle Scholar
  80. 80.
    Lee H, Park JC, Shin SK, et al. Preliminary study of enteroscopy-guided, self-expandable metal stent placement for malignant small bowel obstruction. J Gastroenterol Hepatol. 2012;27(7):1181–6.PubMedGoogle Scholar
  81. 81.
    Fukumoto A, Manabe N, Tanaka S, et al. Usefulness of EUS with double-balloon enteroscopy for diagnosis of small-bowel diseases. Gastrointest Endosc. 2007;65(3):412–20.PubMedGoogle Scholar
  82. 82.
    Ivanova E, Fedorov ED, Budzinskiy S, et al. Which lesions found by balloon-assisted enteroscopy should undergo EUS examination and benefit from it? Gastrointest Endosc. 2013;77(5S):AB421.Google Scholar
  83. 83.
    Boudiaf M, Jaff A, Soyer P, et al. Small-bowel diseases: prospective evaluation of multi-detector row helical CT enteroclysis in 107 consecutive patients. Radiology. 2004;233:338–44.PubMedGoogle Scholar
  84. 84.
    Kamaoui I, De-Luca V, Ficarelli S, et al. Value of CT enteroclysis in suspected small-bowel carcinoid tumors. AJR Am J Roentgenol. 2010;194:629–33.PubMedGoogle Scholar
  85. 85.
    Van Weyenberg SJ, Meijerink MR, Jacobs MA, et al. MR enteroclysis in the diagnosis of small-bowel neoplasms. Radiology. 2010;254:765–73.PubMedGoogle Scholar
  86. 86.
    Brosens LAA, Jansen M, Giardiello FM, Offerhaus GJA. Polyps of the small intestine. Diagn Histopathol. 2011;17(2):69–79.Google Scholar
  87. 87.
    Culver EL, McIntyre AS. Sporadic duodenal polyps: classification, investigation and management. Endoscopy. 2011;43:144–55.PubMedGoogle Scholar
  88. 88.
    Jepsen JM, Persson M, Jakobsen NO, et al. Prospective study of prevalence and endoscopic and histopathologic characteristics of duodenal polyps in patients submitted to upper endoscopy. Scand J Gastroenterol. 1994;29(6):483–7.PubMedGoogle Scholar
  89. 89.
    Lépilliez V, Chemaly M, Ponchon T, et al. Endoscopic resection of sporadic duodenal adenomas: an efficient technique with a substantial risk of delayed bleeding. Endoscopy. 2008;40(10):806–10.PubMedGoogle Scholar
  90. 90.
    van Heumen BW, Mul K, Nagtegaal ID, et al. Management of sporadic duodenal adenomas and the association with colorectal neoplasms: a retrospective cohort study. J Clin Gastroenterol. 2012;46(5):390–6.PubMedGoogle Scholar
  91. 91.
    Murray MA, Zimmerman MJ, Ee HC. Sporadic duodenal adenoma is associated with colorectal neoplasia. Gut. 2004;53(2):261–5.PubMedCentralPubMedGoogle Scholar
  92. 92.
    Arber N, Moshkowitz M. Small bowel polyposis syndromes. Curr Gastroenterol Rep. 2011;13(5):435–41.PubMedGoogle Scholar
  93. 93.
    Bulow S, Bjork J, Christensen IJ, et al. Duodenal adenomatosis in familial adenomatous polyposis. Gut. 2004;53:381–6.PubMedCentralPubMedGoogle Scholar
  94. 94.
    Groves CJ, Saunders BP, Spigelman AD, et al. Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study. Gut. 2002;50:636–41.PubMedCentralPubMedGoogle Scholar
  95. 95.
    Saurin JC, Gutknecht C, Napoleon B, et al. Surveillance of duodenal adenomas in familial adenomatous polyposis reveals high cumulative risk of advanced disease. J Clin Oncol. 2004;22:493–8.PubMedGoogle Scholar
  96. 96.
    Schulz AC, Bojarski C, Buhr HJ, Kroesen AJ. Occurrence of adenomas in the pouch and small intestine of FAP patients after proctocolectomy with ileoanal pouch construction. Int J Colorectal Dis. 2008;23(4):437–41.PubMedGoogle Scholar
  97. 97.
    Sampson JR, Jones N. MUTYH-associated polyposis. Best Pract Res Clin Gastroenterol. 2009;23(2):209–18.PubMedGoogle Scholar
  98. 98.
    Vasen HF, Möslein G, Alonso A, et al. Guidelines for the clinical management of familial adenomatous polyposis (FAP). Gut. 2008;57(5):704–13.PubMedGoogle Scholar
  99. 99.
    Utsunomiya J, Gocho H, Miyanaga T, et al. Peutz-Jeghers syndrome: its natural course and management. Johns Hopkins Med J. 1975;136:71–82.PubMedGoogle Scholar
  100. 100.
    Latchford AR, Phillips RK. Gastrointestinal polyps and cancer in Peutz-Jeghers syndrome: clinical aspects. Fam Cancer. 2011;10(3):455–61.PubMedGoogle Scholar
  101. 101.
    Giardiello FM, Brensinger JD, Tersmette AC, et al. Very high risk of cancer in familial Peutz-Jeghers syndrome. Gastroenterology. 2000;119:1447–53.PubMedGoogle Scholar
  102. 102.
    Boardman LA, Thibodeau SN, Schaid DJ, et al. Increased risk for cancer in patients with the Peutz-Jeghers syndrome. Ann Intern Med. 1998;128:896–9.PubMedGoogle Scholar
  103. 103.
    Hearle N, Schumacher V, Menko FH, et al. Frequency and spectrum of cancers in the Peutz-Jeghers syndrome. Clin Cancer Res. 2006;12:3209–15.PubMedGoogle Scholar
  104. 104.
    Beggs AD, Latchford AR, Vasen HF, et al. Peutz-Jeghers syndrome: a systematic review and recommendations for management. Gut. 2010;59(7):975–86.PubMedGoogle Scholar
  105. 105.
    Koornstra JJ. Small bowel endoscopy in familial adenomatous polyposis and Lynch syndrome. Best Pract Res Clin Gastroenterol. 2012;26(3):359–68.PubMedGoogle Scholar
  106. 106.
    Saurin JC, Pilleul F, Soussan EB, et al. and the Capsule Commission of the French Society of Digestive Endoscopy (SFED). Small-bowel capsule endoscopy diagnoses early and advanced neoplasms in asymptomatic patients with Lynch syndrome. Endoscopy. 2010;42:1057–62.Google Scholar
  107. 107.
    Tulchinsky H, Keidar A, Strul H, et al. Extracolonic manifestations of familial adenomatous polyposis after proctocolectomy. Arch Surg. 2005;140(2):159–63. discussion 164.PubMedGoogle Scholar
  108. 108.
    Spigelman AD, Williams CB, Talbot IC, et al. Upper gastrointestinal cancer in patients with familial adenomatous polyposis. Lancet. 1989;2:783–5.PubMedGoogle Scholar
  109. 109.
    Mathus-Vliegen EM, Boparai KS, Dekker E, van Geloven N. Progression of duodenal adenomatosis in familial adenomatous polyposis: due to ageing of subjects and advances in technology. Fam Cancer. 2011;10(3):491–9.PubMedCentralPubMedGoogle Scholar
  110. 110.
    Burke CA, Beck GJ, Church JM, van Stolk RU. The natural history of untreated duodenal and ampullary adenomas in patients with familial adenomatous polyposis followed in an endoscopic surveillance program. Gastrointest Endosc. 1999;49:358–64.PubMedGoogle Scholar
  111. 111.
    Matsumoto T. Natural history of ampullary adenoma in familial adenomatous polyposis: reconfirmation of benign nature during extended surveillance. Am J Gastroenterol. 2000;95:1557–62.PubMedGoogle Scholar
  112. 112.
    Mönkemüller K, Fry LC, Ebert M, et al. Feasibility of double-balloon enteroscopy-assisted chromoendoscopy of the small bowel in patients with familial adenomatous polyposis. Endoscopy. 2007;39:52–7.PubMedGoogle Scholar
  113. 113.
    Tajika M, Niwa Y, Bhatia V, et al. Risk of ileal pouch neoplasms in patients with familial adenomatous polyposis. World J Gastroenterol. 2013;19(40):6774–83.PubMedCentralPubMedGoogle Scholar
  114. 114.
    Brosens LA, Keller JJ, Offerhaus GJ, et al. Prevention and management of duodenal polyps in familial adenomatous polyposis. Gut. 2005;54:1034–43.PubMedCentralPubMedGoogle Scholar
  115. 115.
    Alderlieste YA, Bastiaansen BA, Mathus-Vliegen EM, et al. High rate of recurrent adenomatosis during endoscopic surveillance after duodenectomy in patients with familial adenomatous polyposis. Fam Cancer. 2013;12(4):699–706.PubMedGoogle Scholar
  116. 116.
    Moussata D, Nancey S, Lapalus MG. Frequency and severity of ileal adenomas in familial adenomatous polyposis after colectomy. Endoscopy. 2008;40(2):120–5.PubMedGoogle Scholar
  117. 117.
    Saurin JC, Napoleon B, Gay G, et al. Endoscopic management of patients with familial adenomatous polyposis (FAP) following a colectomy. Endoscopy. 2005;37:499–501.PubMedGoogle Scholar
  118. 118.
    Wong RF, Tuteja AK, Haslem DS, et al. Video capsule endoscopy compared with standard endoscopy for the evaluation of small-bowel polyps in persons with familial adenomatous polyposis (with video). Gastrointest Endosc. 2006;64(4):530–7.PubMedGoogle Scholar
  119. 119.
    Burke CA, Santisi J, Church J, Levinthal G. The utility of capsule endoscopy small bowel surveillance in patients with polyposis. Am J Gastroenterol. 2005;100(7):1498–502.PubMedGoogle Scholar
  120. 120.
    Alderlieste YA, Rauws EA, Mathus-Vliegen EM, et al. Prospective enteroscopic evaluation of jejunal polyposis in patients with familial adenomatous polyposis and advanced duodenal polyposis. Fam Cancer. 2013;12(1):51–6.PubMedGoogle Scholar
  121. 121.
    Mata A, Llach J, Castells A. A prospective trial comparing wireless capsule endoscopy and barium contrast series for small-bowel surveillance in hereditary GI polyposis syndromes. Gastrointest Endosc. 2005;61(6):721–5.PubMedGoogle Scholar
  122. 122.
    Caspari R, von Falkenhausen M, Krautmacher C, et al. Comparison of capsule endoscopy and magnetic resonance imaging for the detection of polyps of the small intestine in patients with familial adenomatous polyposis or with Peutz-Jeghers’ syndrome. Endoscopy. 2004;36(12):1054–9.PubMedGoogle Scholar
  123. 123.
    Tescher P, Macrae FA, Speer T, et al. Surveillance of FAP: a prospective blinded comparison of capsule endoscopy and other GI imaging to detect small bowel polyps. Hered Cancer Clin Pract. 2010;8(1):3.PubMedCentralPubMedGoogle Scholar
  124. 124.
    Matsumoto T, Esaki M, Yanaru-Fujisawa R, et al. Small-intestinal involvement in familial adenomatous polyposis: evaluation by double-balloon endoscopy and intraoperative enteroscopy. Gastrointest Endosc. 2008;68(5):911–9.PubMedGoogle Scholar
  125. 125.
    Ruys AT, Alderlieste YA, Gouma DJ, et al. Jejunal cancer in patients with familial adenomatous polyposis. Clin Gastroenterol Hepatol. 2010;8(8):731–3.PubMedGoogle Scholar
  126. 126.
    Will OC, Man RF, Phillips RK, et al. Familial adenomatous polyposis and the small bowel: a loco-regional review and current management strategies. Pathol Res Pract. 2008;204(7):449–58.PubMedGoogle Scholar
  127. 127.
    Mönkemüller K, Bellutti M, Fry LC, Malfertheiner P. Enteroscopy. Best Pract Res Clin Gastroenterol. 2008;22(5):789–811.PubMedGoogle Scholar
  128. 128.
    Bellutti M, Fry LC, Malfertheiner P, Mönkemüller K. Utility of double balloon enteroscopy for surveillance in intestinal polyposis syndromes. Tech Gastrointest Endosc. 2008;10(3):96–100.Google Scholar
  129. 129.
    Marmo C, Bizzotto A, Riccioni ME, et al. Multicentric italian study that compares diagnostic and prognostic capacity of balloon-assisted enteroscopy (BAE) and videocaspule endoscopy in patients with familial polyposis: preliminary results. Abstracts of the 20th National Congress of Digestive Diseases, Italian Federation of Societies of Digestive Diseases – FISMAD. Dig Liver Dis. 2014;46(S2):S130.Google Scholar
  130. 130.
    Groen EJ, Roos A, Muntinghe FL, et al. Extra-intestinal manifestations of familial adenomatous polyposis. Ann Surg Oncol. 2008;15(9):2439–50.PubMedCentralPubMedGoogle Scholar
  131. 131.
    Casillas J, Sais GJ, Greve JL, et al. Imaging of intra- and extraabdominal desmoid tumors. Radiographics. 1991;11:959–68.PubMedGoogle Scholar
  132. 132.
    Vandevenne JE, De Schepper AM, De Beuckeleer L, et al. New concepts in understanding evolution of desmoid tumors: MR imaging of 30 lesions. Eur Radiol. 1997;7:1013–9.PubMedGoogle Scholar
  133. 133.
    Hinds R, Philp C, Hyer W, Fell JM. Complications of childhood Peutz-Jeghers syndrome: implications for pediatric screening. J Pediatr Gastroenterol Nutr. 2004;39(2):219–20.PubMedGoogle Scholar
  134. 134.
    van Lier MG, Mathus-Vliegen EM, Wagner A, et al. High cumulative risk of intussusception in patients with Peutz-Jeghers syndrome: time to update surveillance guidelines? Am J Gastroenterol. 2011;106(5):940–5.PubMedGoogle Scholar
  135. 135.
    Parsi MA, Burke CA. Utility of capsule endoscopy in Peutz-Jeghers syndrome. Gastrointest Endosc Clin N Am. 2004;14(1):159–67.PubMedGoogle Scholar
  136. 136.
    van Lier MG, Wagner A, Mathus-Vliegen EM. High cancer risk in Peutz-Jeghers syndrome: a systematic review and surveillance recommendations. Am J Gastroenterol. 2010;105(6):1258–64.PubMedGoogle Scholar
  137. 137.
    Korsse SE, Dewint P, Kuipers EJ, van Leerdam ME. Small bowel endoscopy and Peutz-Jeghers syndrome. Best Pract Res Clin Gastroenterol. 2012;26(3):263–78.PubMedGoogle Scholar
  138. 138.
    Pennazio M, Rossini FP. Small bowel polyps in Peutz-Jeghers syndrome: management by combined push enteroscopy and intraoperative enteroscopy. Gastrointest Endosc. 2000;51(3):304–8.PubMedGoogle Scholar
  139. 139.
    Brown G, Fraser C, Schofield G, et al. Video capsule endoscopy in peutz-jeghers syndrome: a blinded comparison with barium follow-through for detection of small-bowel polyps. Endoscopy. 2006;38(4):385–90.PubMedGoogle Scholar
  140. 140.
    Ohmiya N, Taguchi A, Shirai K, et al. Endoscopic resection of Peutz-Jeghers polyps throughout the small intestine at double-balloon enteroscopy without laparotomy. Gastrointest Endosc. 2005;61(1):140–7.PubMedGoogle Scholar
  141. 141.
    Ohmiya N, Nakamura M, Takenaka H, et al. Management of small-bowel polyps in Peutz-Jeghers syndrome by using enteroclysis, double-balloon enteroscopy, and videocapsule endoscopy. Gastrointest Endosc. 2010;72(6):1209–16.PubMedGoogle Scholar
  142. 142.
    Gao H, van Lier MG, Poley JW, et al. Endoscopic therapy of small-bowel polyps by double-balloon enteroscopy in patients with Peutz-Jeghers syndrome. Gastrointest Endosc. 2010;71(4):768–73.PubMedGoogle Scholar
  143. 143.
    Sakamoto H, Yamamoto H, Hayashi Y, et al. Nonsurgical management of small-bowel polyps in Peutz-Jeghers syndrome with extensive polypectomy by using double-balloon endoscopy. Gastrointest Endosc. 2011;74(2):328–33.PubMedGoogle Scholar
  144. 144.
    Chen TH, Lin WP, Su MY, et al. Balloon-assisted enteroscopy with prophylactic polypectomy for Peutz-Jeghers syndrome: experience in Taiwan. Dig Dis Sci. 2011;56(5):1472–5.PubMedGoogle Scholar
  145. 145.
    Serrano M, Mão-de-Ferro S, Pinho R, et al. Double-balloon enteroscopy in the management of patients with Peutz-Jeghers syndrome: a retrospective color multicenter study. Rev Esp Enferm Dig. 2013;105(10):594–9.PubMedGoogle Scholar
  146. 146.
    Lin TK. Enteroscopy in the pediatric population. Tech Gastrointest Endosc. 2013;15(1):36–40.Google Scholar
  147. 147.
    May A, Nachbar L, Pohl J, Ell C. Endoscopic interventions in the small bowel using double balloon enteroscopy: feasibility and limitations. Am J Gastroenterol. 2007;102(3):527–35.PubMedGoogle Scholar
  148. 148.
    Mathus-Vliegen EM, Tytgat GN. Intraoperative endoscopy: technique, indications, and results. Gastrointest Endosc. 1986;32(6):381–4.PubMedGoogle Scholar

Copyright information

© Springer International Publishing Switzerland 2015

Authors and Affiliations

  • Alessandra Bizzotto
    • 1
  • Maria Elena Riccioni
    • 1
    Email author
  • Rosario Landi
    • 1
  • Clelia Marmo
    • 1
  • Brunella Barbaro
    • 2
  • Guido Costamagna
    • 1
  1. 1.Digestive Endoscopy UnitUniversità Cattolica del Sacro CuoreRomeItaly
  2. 2.Department of Bioimaging and Radiological SciencesUniversità Cattolica del Sacro CuoreRomeItaly

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