Current Perspectives on Small Bowel Tumors: Overview of Prevalence, Clinical Manifestations, and Treatment Approaches

Thitichai Wongsiriamnuey; Julajak Limsrivilai

doi:10.33192/smj.v76i4.267555

Authors

Thitichai Wongsiriamnuey Mahidol Bamrungrak Nakhonsawan Medical Center, Mahidol University Nakhonsawan Campus, Nakhonsawan, Thailand
Julajak Limsrivilai Division of Gastroenterology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand

DOI:

https://doi.org/10.33192/smj.v76i4.267555

Keywords:

Small bowel tumors, GIST, NET, lymphoma, neuroendocrine, gastrointestinal stromal

Abstract

Small bowel tumors (SBTs) constitute a rare yet increasingly recognized group of gastrointestinal neoplasms, accounting for less than 5% of all gastrointestinal cancers. Despite their infrequency, the incidence of SBTs has exhibited a notable upward trend, underscoring the importance of understanding these diverse and complex tumors. This review consolidates current knowledge on SBTs, encompassing epidemiology, risk factors, clinical manifestations, diagnostic advancements, and treatment modalities. Data from various sources are analyzed to present a comprehensive overview of the evolving landscape of SBTs. Our findings indicate that adenocarcinomas, carcinoid tumors, lymphomas, and gastrointestinal stromal tumors (GISTs) are the common SBTs. While adenocarcinoma and neuroendocrine tumors are the common types of SBTs in the West, GIST and lymphoma are more common in Asia. Common risk factors include genetic syndromes and inflammatory bowel diseases. There is variability in clinical presentations depending on the type of tumors. Although diagnostic challenges persist, advancements in imaging and endoscopic techniques have improved detection rates. Treatment strategies are evolving; surgical resection remains the mainstay for localized disease, augmented by systemic therapies and targeted agents for advanced stages. This review emphasizes the importance of early detection and individualized treatment approaches in improving outcomes for SBT patients. It addresses the need for ongoing research and innovation in managing these tumors.

References

Siegel RL, Miller KD, Fuchs HE, Jemal A. Cancer Statistics, 2021. CA Cancer J Clin. 2021;71(1):7-33.

Pourmand K, Itzkowitz SH. Small Bowel Neoplasms and Polyps. Curr Gastroenterol Rep. 2016;18(5):23.

Yao H, Sokas C, Welch HG. Rising Incidence of Cancer of the Small Intestine: Overdiagnosis and Better Diagnosis of Low-lethality Disease. Gastroenterology. 2022;162(6):1749-51.

Rondonotti E, Koulaouzidis A, Georgiou J, Pennazio M. Small bowel tumours, update in diagnosis and management. Curr Opin Gastroenterol. 2018;34(3):159-64.

Liao Z, Gao R, Xu C, Li Z. Indications and detection, completion, and retention rates of small-bowel capsule endoscopy: a systematic review. Gastrointest Endosc. 2010;71(2):280-6.

Miller G, Boman J, Shrier I, Gordon PH. Etiology of small bowel obstruction. The Am J Surg. 2000;180(1):33-6.

Bilimoria KY, Bentrem DJ, Wayne JD, Ko CY, Bennett CL, Talamonti MS. 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.

Bouvier AM, Robaszkiewicz M, Jooste V, Cariou M, Drouillard A, Bouvier V, et al. Trends in incidence of small bowel cancer according to histology: a population-based study. J Gastroenterol. 2020;55(2):181-8.

Tangkittikasem N, Boonyaarunnate T, Aswakul P, Kachintorn U, Prachayakul V. Clinical, Radiologic, and Endoscopic Manifestations of Small Bowel Malignancies: a First Report from Thailand. Asian Pac J Cancer Prev. 2015;16(18):8613-8.

Chung CS, Tai CM, Huang TY, Chang CW, Chen KC, Tseng CM, et al. Small bowel tumors: A digestive endoscopy society of Taiwan (DEST) multicenter enteroscopy-based epidemiologic study. J Formos Med Assoc. 2018;117(8):705-10.

Uchino M, Ikeuchi H, Hata K, Minagawa T, Horio Y, Kuwahara R, et al. Intestinal cancer in patients with Crohn's disease: A systematic review and meta-analysis. J Gastroenterol Hepatol. 2021;36(2):329-36.

Barsouk A, Rawla P, Barsouk A, Thandra KC. Epidemiology of Cancers of the Small Intestine: Trends, Risk Factors, and Prevention. Med Sci (Basel). 2019;7(3):46.

Chin YH, Jain SR, Lee MH, Ng CH, Lin SY, Mai AS, 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(1):239-50.

Yazdanpanah O, Surapaneni S, Shanah L, Kabashneh S. Diagnosis and Management of Gastrointestinal Neuroendocrine Tumors: A Comprehensive Literature Review. Cureus. 2021;13(3):e14006.

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

Hatzaras I, Palesty JA, Abir F, Sullivan P, Kozol RA, Dudrick SJ, Longo WE. Small-bowel tumors: epidemiologic and clinical characteristics of 1260 cases from the connecticut tumor registry. Arch Surg. 2007;142(3):229-35.

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.

Ojha A, Zacherl J, Scheuba C, Jakesz R, Wenzl E. Primary small bowel malignancies: single-center results of three decades. J Clin Gastroenterol. 2000;30(3):289-93.

Shinya T. Malignant Small Bowel Neoplasms:a review of post-contrast multiphasic multidetector computed tomography. J Med Invest. 2022;69(1.2):19-24.

Rondonotti E, Pennazio M, Toth E, Menchen P, Riccioni ME, De Palma GD, et al. Small-bowel neoplasms in patients undergoing video capsule endoscopy: a multicenter European study. Endoscopy. 2008;40(6):488-95.

Cheung DY, Lee I-S, Chang DK, Kim JO, Cheon JH, Jang BI, et al. Capsule endoscopy in small bowel tumors: A multicenter Korean study. J Gastroenterol Hepatol. 2010;25(6):1079-86.

Lewis BS, Eisen GM, Friedman S. A pooled analysis to evaluate results of capsule endoscopy trials. Endoscopy. 2005;37(10):960-5.

Pennazio M, Rondonotti E, de Franchis R. Capsule endoscopy in neoplastic diseases. World J Gastroenterol. 2008;14(34):5245-53.

Limsrivilai J, Srisajjakul S, Pongprasobchai S, Leelakusolvong S, Tanwandee T. A Prospective Blinded Comparison of Video Capsule Endoscopy Versus Computed Tomography Enterography in Potential Small Bowel Bleeding: Clinical Utility of Computed Tomography Enterography. J Clin Gastroenterol. 2017;51(7):611-8.

ASGE Standards of Practice Committee, Gurudu SR, Bruining DH, Acosta RD, Eloubeidi MA, Faulx AL, et al. The role of endoscopy in the management of suspected small-bowel bleeding. Gastrointest Endosc. 2017;85(1):22-31.

Tran CG, Sherman SK, Howe JR. Small Bowel Neuroendocrine Tumors. Curr Probl Surg. 2020;57(12):100823.

Ahmed M. Gastrointestinal neuroendocrine tumor in 2020. World J Gastrointest Oncol. 2020;12(8):791-807.

Oberg K, Couvelard A, Delle Fave G, Gross D, Grossman A, Jensen RT, et al. ENETS Consensus Guidelines for Standard of Care in Neuroendocrine Tumours: Biochemical Markers. Neuroendocrinology. 2017;105(3):201-11.

Khosla D, Dey T, Madan R, Gupta R, Goyal S, Kumar N, Kapoor R. Small bowel adenocarcinoma: An overview. World Journal of Gastrointestinal Oncology. World J Gastrointest Oncol. 2022;14(2):413-22.

Marabelle A, Le DT, Ascierto PA, Di Giacomo AM, De Jesus-Acosta A, Delord JP, et al. Efficacy of Pembrolizumab in Patients With Noncolorectal High Microsatellite Instability/Mismatch Repair-Deficient Cancer: Results From the Phase II KEYNOTE-158 Study. J Clin Oncol. 2020;38(1):1-10.

Pedersen KS, Foster NR, Overman MJ, Boland PM, Kim SS, Arrambide KA, et al. ZEBRA: A Multicenter Phase II Study of Pembrolizumab in Patients with Advanced Small-Bowel Adenocarcinoma. Clin Cancer Res. 2021;27(13):3641-8.

Casali PG, Blay JY, Abecassis N, Bajpai J, Bauer S, Biagini R, et al. Gastrointestinal stromal tumours: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2022;33(1):20-33.

Demetri GD, van Oosterom AT, Garrett CR, Blackstein ME, Shah MH, Verweij J, et al. Efficacy and safety of sunitinib in patients with advanced gastrointestinal stromal tumour after failure of imatinib: a randomised controlled trial. Lancet. 2006;368(9544):1329-38.

Prempree T, Wongpaksa C. cKIT Mutations in GISTs and Other Malignancies in Thailand. Siriraj Med J [Internet]. 2006 Mar. 1 [cited 2024 Feb. 19];58(3):711-5. Available from: https://he02.tci-thaijo.org/index.php/sirirajmedj/article/view/245726

Von Mehren M, Kane JM, Riedel RF, Sicklick JK, Pollack SM, Agulnik M, et al. NCCN Guidelines® Insights: Gastrointestinal Stromal Tumors, Version 2.2022. J Natl Compr Canc Netw. 2022;20(11):1204-14.