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Small Bowel Cancer

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Abdominal Cancer Alliance

What is Small Bowel Cancer
(Adenocarcinoma)?

Small bowel cancers are extremely rare, accounting for less than 5% of gastrointestinal cancers and less than 1% of all cancers. Because the small bowel is difficult to examine with standard screening tools, these cancers are often diagnosed at advanced stages—over 30% have already spread, most commonly to the liver or peritoneal cavity.  

The Basics

While there is abundant information on more conventional treatments for Small Bowel Cancer, our focus here will be on the rationale and available data supporting the use of cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) in these specific cases. It is important to emphasize that evidence regarding the benefit of CRS/HIPEC for Small Bowel Cancer remains limited. Nevertheless, reports suggest potential benefits for certain patients facing Small Bowel Cancer with rare indications for CRS/HIPEC.

 

Small bowel cancers are very rare, making up <5% of all gastrointestinal cancers and <1% of all cancers.[see References: 1] These cancers include many different histologic types, with adenocarcinomas being the most common, accounting for about 40% of cases.[2] Adenocarcinomas are a type of cancer that starts in the glands that line your organs.  Unlike colon cancer, which can be detected early through colonoscopy, screening for small bowel cancer is much more challenging due to difficulty in accessing the small bowel with endoscopy. As a result, most small bowel cancers are diagnosed at an advanced stages.[3-5] More than 32% of these cancers have spread to other parts of the body by the time they are diagnosed, most commonly to the liver (59%) and the peritoneal cavity (25%).[6] Once the cancer has spread, the chances of survival decrease, with a 5-year survival rate of <20%.[3]

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Treatment for small bowel cancer typically involves surgery to remove the affected part of the small bowel, along with nearby lymph nodes and some of the tissue that attaches the small bowel to the abdominal wall. Chemotherapy or radiation therapy may be used before or after surgery to shrink the tumor or treat any residual cancer cells. The treatment plan depends on how far the cancer has spread and whether the tumor can be fully removed through surgery. There is no clear consensus on the optimal treatment for metastatic small bowel cancers. Traditionally, patients are treated with palliative intent using 5-FU or oxaliplatin-based chemotherapy to manage symptoms and slow disease progression. Unfortunately, the prognosis with this approach is poor, with a median survival ranging from 9-16 months. [7-10]

Currently, there are no established guidelines for treating peritoneal metastases from small bowel cancer. However, because cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (CRS/HIPEC) has been successful in treating colorectal cancers and other similar cancers with peritoneal metastases,[11, 12] physicians are beginning to explore its use in small bowel cancer.[13] Although there are very few available studies on this approach, evidence suggests that CRS/HIPEC could improve survival in highly selected patients, with survival rates approaching those seen in colorectal cancer with peritoneal metastases.[14] The largest multi-institutional study, which included 152 patients with small bowel cancer treated with CRS/HIPEC, found a median overall survival of 32 months and a median progression free survival of 14 months.[15] Other smaller studies report median overall survival ranging from 9-32 months.[14] Most patients in these studies also received systemic chemotherapy (chemotherapy given intravenously) before and/or after the CRS/HIPEC procedure.[15-17] All studies emphasize the importance of carefully selecting patients based on factors such as ability to achieve complete tumor removal, tumor burden (PCI score), the presence of lymph node metastases, and tumor grade, as these significantly affect survival outcomes.[15, 16] However, the available data is extremely limited and additional prospective studies are needed to identify which patients will benefit most from this approach.

There have been no studies directly comparing CRS/HIPEC to alternative treatments for small bowel cancer with peritoneal metastases. However, one meta-analysis compared outcomes of patients treated with either systemic chemotherapy or CRS with intraperitoneal chemotherapy and concluded that CRS with intraperitoneal chemotherapy appears to be more effective than systemic chemotherapy alone. The median overall survival of patients treated with CRS and intraperitoneal chemotherapy ranged from 16-27 months, while those treated with systemic chemotherapy alone had a median survival of 8-20 months.[18] In contrast, patients who received only supportive care had a survival of <3 months.[19] While the survival differences are promising, it is important to note that recurrence after CRS/HIPEC is common. Additionally, the patient populations in these studies are diverse, which makes it difficult to directly compare outcomes across different studies.[14] Therefore, CRS/HIPEC may be an effective treatment option for certain patients, but should only be considered at specialized centers focused on peritoneal surface malignancies.

Finding Specialized Care

Finding a care provider who is knowledgeable with colorectal cancer and how to treat it is critically important. To search for providers who specialize in performing CRS/HIPEC and providers who specialize in colorectal cancer, navigate to our Find a Specialist page.​​

Please note: Our goal is to provide information to help you find a doctor closest to your home that can provide the best quality of care for your diagnosis or your anticipated CRS/HIPEC procedure.  The Abdominal Cancers Alliance does not endorse any care provider or medical center over another.

Research

Clinical Trials

There are many actively recruiting clinical trials for small bowel cancer registered online. A full list of all active clinical trials and their current recruitment status can be found on www.ClinicalTrials.gov. Your oncologists (medical, surgical, and radiation) can also help you review clinical trial options and recommendations.  There are currently no clinical trials for HIPEC in small bowel, probably due to the rarity cancer itself.
 

Other Resources

More Information on Small Bowel Cancer
American Cancer Society
National Cancer Institute
Patient Support
GI Cancers Alliance

Facing cancer is hard.
But you are not alone - we’re right here with you.

Helping Hands
Patient and Caregiver Network
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Stories of hope

References

1. Siegel RL, Miller KD, Jemal A. Cancer statistics, 2020. CA Cancer J Clin. 2020 Jan;70(1):7-30. doi: 10.3322/caac.21590. Epub 2020 Jan 8. PMID: 31912902. 2. Barsouk A, Rawla P, Barsouk A, Thandra KC. Epidemiology of Cancers of the Small Intestine: Trends, Risk Factors, and Prevention. Med Sci (Basel). 2019 Mar 17;7(3):46. doi: 10.3390/medsci7030046. PMID: 30884915; PMCID: PMC6473503. 3. Young JI, Mongoue-Tchokote S, Wieghard N, Mori M, Vaccaro GM, Sheppard BC, Tsikitis VL. Treatment and Survival of Small-bowel Adenocarcinoma in the United States: A Comparison With Colon Cancer. Dis Colon Rectum. 2016 Apr;59(4):306-15. doi: 10.1097/DCR.0000000000000562. PMID: 26953989. 4. Pedersen KS, Raghav K, Overman MJ. Small Bowel Adenocarcinoma: Etiology, Presentation, and Molecular Alterations. J Natl Compr Canc Netw. 2019 Sep 1;17(9):1135-1141. doi: 10.6004/jnccn.2019.7344. PMID: 31487680. 5. Akce M, Jiang R, Zakka K, Wu C, Alese OB, Shaib WL, Behera M, El-Rayes BF. Clinical Outcomes of Small Bowel Adenocarcinoma. Clin Colorectal Cancer. 2019 Dec;18(4):257-268. doi: 10.1016/j.clcc.2019.08.002. Epub 2019 Sep 4. PMID: 31606297. 6. 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 Aug 1;101(3):518-26. doi: 10.1002/cncr.20404. PMID: 15274064. 7. Czaykowski P, Hui D. Chemotherapy in small bowel adenocarcinoma: 10-year experience of the British Columbia Cancer Agency. Clin Oncol (R Coll Radiol). 2007 Mar;19(2):143-9. doi: 10.1016/j.clon.2006.12.001. PMID: 17355111. 8. Moon YW, Rha SY, Shin SJ, Chang H, Shim HS, Roh JK. Adenocarcinoma of the small bowel at a single Korean institute: management and prognosticators. J Cancer Res Clin Oncol. 2010 Mar;136(3):387-94. doi: 10.1007/s00432-009-0668-3. Epub 2009 Sep 16. PMID: 19760196. 9. Halfdanarson TR, McWilliams RR, Donohue JH, Quevedo JF. A single-institution experience with 491 cases of small bowel adenocarcinoma. Am J Surg. 2010 Jun;199(6):797-803. doi: 10.1016/j.amjsurg.2009.05.037. PMID: 20609724. 10. Khan K, Peckitt C, Sclafani F, Watkins D, Rao S, Starling N, Jain V, Trivedi S, Stanway S, Cunningham D, Chau I. Prognostic factors and treatment outcomes in patients with Small Bowel Adenocarcinoma (SBA): the Royal Marsden Hospital (RMH) experience. BMC Cancer. 2015 Jan 21;15:15. doi: 10.1186/s12885-015-1014-6. PMID: 25603878; PMCID: PMC4305243. 11. Flood M, Narasimhan V, Waters P, Ramsay R, Michael M, Warrier S, Heriot A. Survival after cytoreductive surgery and hyperthermic intraperitoneal chemotherapy for colorectal peritoneal metastases: A systematic review and discussion of latest controversies. Surgeon. 2021 Oct;19(5):310-320. doi: 10.1016/j.surge.2020.08.016. Epub 2020 Oct 3. PMID: 33023847. 12. van Driel WJ, Koole SN, Sikorska K, Schagen van Leeuwen JH, Schreuder HWR, Hermans RHM, de Hingh IHJT, van der Velden J, Arts HJ, Massuger LFAG, Aalbers AGJ, Verwaal VJ, Kieffer JM, Van de Vijver KK, van Tinteren H, Aaronson NK, Sonke GS. Hyperthermic Intraperitoneal Chemotherapy in Ovarian Cancer. N Engl J Med. 2018 Jan 18;378(3):230-240. doi: 10.1056/NEJMoa1708618. PMID: 29342393. 13. de Bree E, Rovers KP, Stamatiou D, Souglakos J, Michelakis D, de Hingh IH. The evolving management of small bowel adenocarcinoma. Acta Oncol. 2018 Jun;57(6):712-722. doi: 10.1080/0284186X.2018.1433321. Epub 2018 Jan 30. PMID: 29381126. 14. Chen V, Jones M, Cohen L, Yang W, Bedi J, Mohan HM, Apte SS, Larach JT, Flood M, Heriot A, Kong J, Warrier S. Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) in small bowel adenocarcinoma with peritoneal metastasis: a systematic review. Pleura Peritoneum. 2022 Nov 18;7(4):159-167. doi: 10.1515/pp-2022-0121. PMID: 36560970; PMCID: PMC9742456. 15. Liu Y, Yonemura Y, Levine EA, Glehen O, Goere D, Elias D, Morris DL, Sugarbaker PH, Tuech JJ, Cashin P, Spiliotis JD, de Hingh I, Ceelen W, Baumgartner JM, Piso P, Katayama K, Deraco M, Kusamura S, Pocard M, Quenet F, Fushita S; BIG-RENAPE Group. Cytoreductive Surgery Plus Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Metastases From a Small Bowel Adenocarcinoma: Multi-Institutional Experience. Ann Surg Oncol. 2018 May;25(5):1184-1192. doi: 10.1245/s10434-018-6369-x. Epub 2018 Feb 26. PMID: 29484565; PMCID: PMC5891561. 16. Leigh N, Solomon D, Pletcher E, Labow DM, Magge DR, Sarpel U, Golas BJ. The importance of primary tumor origin in gastrointestinal malignancies undergoing cytoreductive surgery and hyperthermic intraperitoneal chemotherapy. World J Surg Oncol. 2020 Jul 23;18(1):182. doi: 10.1186/s12957-020-01938-0. PMID: 32703239; PMCID: PMC7379772. 17. Mah M, Mack LA, Hurton S, Bouchard-Fortier A. Cytoreductive surgery and heated intraperitoneal chemotherapy for peritoneal carcinomatosis from rare etiologies. Am J Surg. 2019 May;217(5):923-927. doi: 10.1016/j.amjsurg.2019.01.011. Epub 2019 Jan 25. PMID: 30760409. 18. Rovers KP, de Bree E, Yonemura Y, de Hingh IH. Treatment of peritoneal metastases from small bowel adenocarcinoma. Int J Hyperthermia. 2017 Aug;33(5):571-578. doi: 10.1080/02656736.2016.1266700. PMID: 27919181. 19. Legué LM, Simkens GA, Creemers GM, Lemmens VEPP, de Hingh IHJT. Synchronous peritoneal metastases of small bowel adenocarcinoma: Insights into an underexposed clinical phenomenon. Eur J Cancer. 2017 Dec;87:84-91. doi: 10.1016/j.ejca.2017.10.012. Epub 2017 Nov 10. PMID: 29132061.

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