Download PDF
Clin Colon Rectal Surg 2024; 37(01): 046-052
DOI: 10.1055/s-0043-1762929
Review Article

Small Bowel Carcinoma in the Setting of Inflammatory Bowel Disease

Himani Bhatt
1   Department of Surgery, Mayo Clinic, Rochester, Minnesota
,
Kellie L. Mathis
1   Department of Surgery, Mayo Clinic, Rochester, Minnesota
› Author Affiliations Funding None.
› Further Information
    Permissions and Reprints

Abstract

Small bowel carcinomas are rare in the general population, but the incidence is increasing. Patients with inflammatory bowel diseases (IBDs) are at significantly higher risk of small bowel adenocarcinomas than their non-IBD counterparts, with Crohn's patients having at least a 12-fold increased risk and ulcerative colitis patients with a more controversial and modest 2-fold increased risk compared with the general population. IBD patients with small bowel carcinomas present with nonspecific symptoms that overlap with typical IBD symptoms, and this results in difficulty making a preoperative diagnosis. Cross-sectional imaging is rarely diagnostic, and most cancers are found incidentally at the time of surgery performed for an IBD indication. As such, most small bowel carcinomas are found at advanced stages and carry a poor prognosis. Oncologic surgical resection is the treatment of choice for patients with locoregional disease with little evidence available to guide adjuvant therapy. Patients with metastatic disease are treated with systemic chemotherapy, and surgery is reserved for palliation in this population. Prognosis is poor with few long-term survivors reported.

Keywords

small bowel adenocarcinoma - small bowel cancer - inflammatory bowel diseases - Crohn's disease - ulcerative colitis


Publication History

Article published online:
15 March 2023

© 2023. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 

  • References

  • 1 Molodecky NA, Soon IS, Rabi DM. et al. Increasing incidence and prevalence of the inflammatory bowel diseases with time, based on systematic review. Gastroenterology 2012; 142 (01) 46-54.e42 , quiz e30
    CrossrefPubMedGoogle Scholar
  • 2 Ng SC, Tang W, Ching JY. et al; Asia–Pacific Crohn's and Colitis Epidemiologic Study (ACCESS) Study Group. Incidence and phenotype of inflammatory bowel disease based on results from the Asia-pacific Crohn's and colitis epidemiology study. Gastroenterology 2013; 145 (01) 158-165.e2
    CrossrefPubMedGoogle Scholar
  • 3 Adami HO, Bretthauer M, Emilsson L. et al. The continuing uncertainty about cancer risk in inflammatory bowel disease. Gut 2016; 65 (06) 889-893
    CrossrefPubMedGoogle Scholar
  • 4 Cahill C, Gordon PH, Petrucci A, Boutros M. Small bowel adenocarcinoma and Crohn's disease: any further ahead than 50 years ago?. World J Gastroenterol 2014; 20 (33) 11486-11495
    CrossrefPubMedGoogle Scholar
  • 5 Piovani D, Hassan C, Repici A. et al. Risk of cancer in inflammatory bowel diseases: umbrella review and reanalysis of meta-analyses. Gastroenterology 2022; 163 (03) 671-684
    CrossrefPubMedGoogle Scholar
  • 6 Eaden JA, Abrams KR, Mayberry JF. The risk of colorectal cancer in ulcerative colitis: a meta-analysis. Gut 2001; 48 (04) 526-535
    CrossrefPubMedGoogle Scholar
  • 7 Kiran RP, Khoury W, Church JM, Lavery IC, Fazio VW, Remzi FH. Colorectal cancer complicating inflammatory bowel disease: similarities and differences between Crohn's and ulcerative colitis based on three decades of experience. Ann Surg 2010; 252 (02) 330-335
    CrossrefPubMedGoogle Scholar
  • 8 Ullman T, Odze R, Farraye FA. Diagnosis and management of dysplasia in patients with ulcerative colitis and Crohn's disease of the colon. Inflamm Bowel Dis 2009; 15 (04) 630-638
    CrossrefPubMedGoogle Scholar
  • 9 Annese V, Daperno M, Rutter MD. et al; European Crohn's and Colitis Organisation. European evidence based consensus for endoscopy in inflammatory bowel disease. J Crohn's Colitis 2013; 7 (12) 982-1018
    CrossrefPubMedGoogle Scholar
  • 10 Bojesen RD, Riis LB, Høgdall E, Nielsen OH, Jess T. Inflammatory bowel disease and small bowel cancer risk, clinical characteristics, and histopathology: a population-based study. Clin Gastroenterol Hepatol 2017; 15 (12) 1900-1907.e2
    CrossrefPubMedGoogle Scholar
  • 11 American Cancer Society. Risk factors for small intestine cancer (adenocarcinoma). Accessed September 14, 2022 at: https://www.cancer.org/cancer/small-intestine-cancer/causes-risks-prevention/risk-factors.html
    PubMed
  • 12 Fields AC, Hu FY, Lu P. et al. Small bowel adenocarcinoma: is there a difference in survival for Crohn's versus sporadic cases?. J Crohn's Colitis 2020; 14 (03) 303-308
    CrossrefPubMedGoogle Scholar
  • 13 Torres J, Mehandru S, Colombel JF, Peyrin-Biroulet L. Crohn's disease. Lancet 2017; 389 (10080): 1741-1755
    CrossrefPubMedGoogle Scholar
  • 14 Clarke WT, Feuerstein JD. Colorectal cancer surveillance in inflammatory bowel disease: practice guidelines and recent developments. World J Gastroenterol 2019; 25 (30) 4148-4157
    CrossrefPubMedGoogle Scholar
  • 15 Lichtenstein GR, Loftus EV, Isaacs KL, Regueiro MD, Gerson LB, Sands BE. ACG clinical guideline: management of Crohn's disease in adults. Am J Gastroenterol 2018; 113 (04) 481-517
    CrossrefPubMedGoogle Scholar
  • 16 Axelrad JE, Olén O, Sachs MC. et al. Inflammatory bowel disease and risk of small bowel cancer: a binational population-based cohort study from Denmark and Sweden. Gut 2021; 70 (02) 297-308
    PubMedGoogle Scholar
  • 17 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 (07) 781-787
    CrossrefPubMedGoogle Scholar
  • 18 American Cancer Society. Key statistics for small intestine cancer. Accessed September 2, 2022 at: https://www.cancer.org/cancer/small-intestine-cancer/about/what-is-key-statistics.html
    PubMed
  • 19 Shaukat A, Virnig DJ, Howard D, Sitaraman SV, Liff JM, Lederle FA. Crohn's disease and small bowel adenocarcinoma: a population-based case-control study. Cancer Epidemiol Biomarkers Prev 2011; 20 (06) 1120-1123
    CrossrefPubMedGoogle Scholar
  • 20 Simon M, Cosnes J, Gornet JM. et al; GETAID group. Endoscopic detection of small bowel dysplasia and adenocarcinoma in Crohn's disease: a prospective cohort-study in high-risk patients. J Crohn's Colitis 2017; 11 (01) 47-52
    CrossrefPubMedGoogle Scholar
  • 21 Kimmel J, Axelrad J. The complex interplay between inflammatory bowel disease and malignancy. Curr Gastroenterol Rep 2020; 22 (03) 13
    CrossrefPubMedGoogle Scholar
  • 22 Kappelman MD, Farkas DK, Long MD. et al. Risk of cancer in patients with inflammatory bowel diseases: a nationwide population-based cohort study with 30 years of follow-up evaluation. Clin Gastroenterol Hepatol 2014; 12 (02) 265-73.e1
    CrossrefPubMedGoogle Scholar
  • 23 Widmar M, Greenstein AJ, Sachar DB, Harpaz N, Bauer JJ, Greenstein AJ. Small bowel adenocarcinoma in Crohn's disease. J Gastrointest Surg 2011; 15 (05) 797-802
    CrossrefPubMedGoogle Scholar
  • 24 Solem CA, Harmsen WS, Zinsmeister AR, Loftus Jr EV. Small intestinal adenocarcinoma in Crohn's disease: a case-control study. Inflamm Bowel Dis 2004; 10 (01) 32-35
    CrossrefPubMedGoogle Scholar
  • 25 von Roon AC, Reese G, Teare J, Constantinides V, Darzi AW, Tekkis PP. The risk of cancer in patients with Crohn's disease. Dis Colon Rectum 2007; 50 (06) 839-855
    CrossrefPubMedGoogle Scholar
  • 26 Menon AM, Mirza AH, Moolla S, Morton DG. Adenocarcinoma of the small bowel arising from a previous strictureplasty for Crohn's disease: report of a case. Dis Colon Rectum 2007; 50 (02) 257-259
    CrossrefPubMedGoogle Scholar
  • 27 Partridge SK, Hodin RA. Small bowel adenocarcinoma at a strictureplasty site in a patient with Crohn's disease: report of a case. Dis Colon Rectum 2004; 47 (05) 778-781
    CrossrefPubMedGoogle Scholar
  • 28 Barwood N, Platell C. Case report: adenocarcinoma arising in a Crohn's stricture of the jejunum. J Gastroenterol Hepatol 1999; 14 (11) 1132-1134
    CrossrefPubMedGoogle Scholar
  • 29 Greenstein AJ. Cancer in inflammatory bowel disease. Mt Sinai J Med 2000; 67 (03) 227-240
    PubMedGoogle Scholar
  • 30 Peyrin-Biroulet L, Ferrante M, Magro F. et al; Scientific Committee of the European Crohn's and Colitis Organization. Results from the 2nd Scientific Workshop of the ECCO. I: impact of mucosal healing on the course of inflammatory bowel disease. J Crohn's Colitis 2011; 5 (05) 477-483
    CrossrefPubMedGoogle Scholar
  • 31 Elriz K, Carrat F, Carbonnel F, Marthey L, Bouvier AM, Beaugerie L. CESAME study group. Incidence, presentation, and prognosis of small bowel adenocarcinoma in patients with small bowel Crohn's disease: a prospective observational study. Inflamm Bowel Dis 2013; 19 (09) 1823-1826
    PubMedGoogle Scholar
  • 32 Weber NK, Fletcher JG, Fidler JL. et al. Clinical characteristics and imaging features of small bowel adenocarcinomas in Crohn's disease. Abdom Imaging 2015; 40 (05) 1060-1067
    CrossrefPubMedGoogle Scholar
  • 33 Floch HF, Slattery LR, Hazzi CG. Carcinoma of the small intestine in regional enteritis: presentation of a case and review of the literature. Am J Gastroenterol 1978; 70 (05) 520-527
    PubMedGoogle Scholar
  • 34 Uchino M, Ikeuchi H, Hata K. et al. Intestinal cancer in patients with Crohn's disease: a systematic review and meta-analysis. J Gastroenterol Hepatol 2021; 36 (02) 329-336
    CrossrefPubMedGoogle Scholar
  • 35 Chin YH, Jain SR, Lee MH. et al. Small bowel adenocarcinoma in Crohn's disease: a systematic review and meta-analysis of the prevalence, manifestation, histopathology, and outcomes. Int J Colorectal Dis 2022; 37 (01) 239-250
    CrossrefPubMedGoogle Scholar
  • 36 Raghav K, Overman MJ. Small bowel adenocarcinomas–existing evidence and evolving paradigms. Nat Rev Clin Oncol 2013; 10 (09) 534-544
    CrossrefPubMedGoogle Scholar
  • 37 Steele SR, Hull TL, Hyman N, Maykel JA, Read TE, Whitlow CB. eds. The ASCRS Textbook of Colon and Rectal Surgery. Springer International Publishing; 2022
    CrossrefGoogle Scholar
  • 38 Reynolds I, Healy P, Mcnamara DA. Malignant tumours of the small intestine. Surgeon 2014; 12 (05) 263-270
    CrossrefPubMedGoogle Scholar
  • 39 Arpa G, Grillo F, Giuffrida P. et al. Separation of low- versus high-grade Crohn's disease-associated small bowel carcinomas is improved by invasive front prognostic marker analysis. J Crohn's Colitis 2020; 14 (03) 295-302
    CrossrefPubMedGoogle Scholar
  • 40 NCCN. Small bowel adenocarcinoma. Accessed September 18, 2022 at: https://www.nccn.org/professionals/physician_gls/pdf/small_bowel.pdf
    PubMed
  • 41 ASGE. Updated guidelines on endoscopic surveillance and management of colorectal dysplasia in inflammatory bowel diseases. Accessed September 11, 2022 at: https://www.asge.org/home/resources/publications/journal-scan/issue/updated-guidelines-on-endoscopic-surveillance-and-management-of-colorectal-dysplasia-in-inflammatory-bowel-diseases
    PubMed
  • 42 Murthy SK, Feuerstein JD, Nguyen GC, Velayos FS. AGA clinical practice update on endoscopic surveillance and management of colorectal dysplasia in inflammatory bowel diseases: expert review. Gastroenterology 2021; 161 (03) 1043-1051.e4
    CrossrefPubMedGoogle Scholar
  • 43 Lamb CA, Kennedy NA, Raine T. et al; IBD guidelines eDelphi consensus group. British Society of Gastroenterology consensus guidelines on the management of inflammatory bowel disease in adults. Gut 2019; 68 (Suppl. 03) s1-s106
    CrossrefPubMedGoogle Scholar
  • 44 American Cancer Society. Tests for small intestine cancer (adenocarcinoma). Accessed September 14, 2022 at: https://www.cancer.org/cancer/small-intestine-cancer/detection-diagnosis-staging/how-diagnosed.html
    PubMed
  • 45 Leal-Valdivieso C, Marín I, Mañosa M. et al. Should we monitor Crohn's disease patients for postoperative recurrence after permanent ileostomy?. Inflamm Bowel Dis 2012; 18 (01) E196
    CrossrefPubMedGoogle Scholar
  • 46 American Cancer Society. Surgery for small intestine cancer (adenocarcinoma). Accessed September 14, 2022 at: https://www.cancer.org/cancer/small-intestine-cancer/treating/surgery.html
    PubMedGoogle Scholar
  • 47 Dabaja BS, Suki D, Pro B, Bonnen M, Ajani J. Adenocarcinoma of the small bowel: presentation, prognostic factors, and outcome of 217 patients. Cancer 2004; 101 (03) 518-526
    CrossrefPubMedGoogle Scholar
  • 48 NCCN. sclc.pdf. Published online 2022. Accessed February 21, 2023 at: https://www.nccn.org/professionals/physician_gls/pdf/survivorship.pdf
    PubMed
  • 49 Annese V, Beaugerie L, Egan L. et al; ECCO. European evidence-based consensus: inflammatory bowel disease and malignancies. J Crohn's Colitis 2015; 9 (11) 945-965
    CrossrefPubMedGoogle Scholar
  • 50 Beaugerie L, Carrat F, Colombel JF. et al; CESAME Study Group. Risk of new or recurrent cancer under immunosuppressive therapy in patients with IBD and previous cancer. Gut 2014; 63 (09) 1416-1423
    CrossrefPubMedGoogle Scholar
  • 51 Lin SC, Goldowsky A, Papamichael K, Cheifetz AS. The treatment of inflammatory bowel disease in patients with a history of malignancy. Inflamm Bowel Dis 2019; 25 (06) 998-1005
    CrossrefPubMedGoogle Scholar
  • 52 Morada AO, Senapathi SH, Bashiri A, Chai S, Cagir B. A systematic review of primary ileostomy site malignancies. Surg Endosc 2022; 36 (03) 1750-1760
    CrossrefPubMedGoogle Scholar
  • 53 Sigler L, Jedd FL. Adenocarcinoma of the ileostomy occurring after colectomy for ulcerative colitis: report of a case. Dis Colon Rectum 1969; 12 (01) 45-48
    CrossrefPubMedGoogle Scholar
  • 54 Mimura T, Kuramoto S, Yamasaki K, Kaminishi M. Familial adenomatous polyposis: a case report and histologic mucin study. J Clin Gastroenterol 1999; 28 (04) 372-376
    CrossrefPubMedGoogle Scholar
  • 55 Gadacz TR, McFadden DW, Gabrielson EW, Ullah A, Berman JJ. Adenocarcinoma of the ileostomy: the latent risk of cancer after colectomy for ulcerative colitis and familial polyposis. Surgery 1990; 107 (06) 698-703
    PubMedGoogle Scholar
  • 56 Attanoos R, Billings PJ, Hughes LE, Williams GT. Ileostomy polyps, adenomas, and adenocarcinomas. Gut 1995; 37 (06) 840-844
    CrossrefPubMedGoogle Scholar
  • 57 Yousaf A, Beatty C, Zinn Z, Cassim R, Gayam S. Ileostomy adenocarcinoma in Crohn's disease. Int J Colorectal Dis 2020; 35 (05) 963-966
    CrossrefPubMedGoogle Scholar