Guideline sources The following summarized guidelines for the evaluation and management of small bowel adenocarcinoma are prepared by our editorial team based on guidelines from the European Society of Gastrointestinal Endoscopy (ESGE 2023), the Society for Immunotherapy of Cancer (SITC 2023), the Japanese Society of Hepato-Biliary-Pancreatic Surgery (JSHBPS/JGCA 2022), the American Society for Gastrointestinal Endoscopy (ASGE 2021), and the French Society of Oncological Radiotherapy (SFRO/GERCOR/UNICANCER/SFCD/SNFGE/FFCD/SFED 2018). 1 2 3 4 5 Calculator Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s TNM class Guidelines https://web.pathway.md/diseases/recaumaGTpYLUoddL 1/5 6/24/23, 3:13 PM Small bowel adenocarcinoma Pathway 1. Classification and risk stratification Risk factors: recognize that there are no known risk factors for non-ampullary duodenal cancer other than familial adenomatous polyposis of the colon. 2. Diagnostic investigations Initial evaluation: Include the following in the pre-treatment evaluation: physical examination CT of the chest, abdomen and pelvis for tumor localization and extension upper and lower gastrointestinal endoscopy of lesions associated with a predisposing disease. E Obtain ultrasound for duodenal sites to determine the possibility of tumor resection in the absence of metastasis. E Imaging for staging: As per ESGE 2023 guidelines, obtain cross-sectional imaging for staging and ascertaining operability in patients with a small bowel capsule endoscopy finding of a small bowel tumor with high diagnostic certainty. B As per JGCA 2022 guidelines, consider obtaining imaging, including contrast-enhanced CT, for the diagnosis of distant metastases. C Genetic testing: Obtain MSI testing in patients with small bowel cancer, including unresectable or recurrent duodenal cancer. B Avoid obtaining HER2 and RAS gene tests in patients with small bowel cancer, including unresectable or recurrent duodenal cancer. D 3. Diagnostic procedures Small bowel endoscopy: Consider performing device-assisted enteroscopy in preference to small bowel capsule endoscopy if imaging has already demonstrated a suspected small bowel tumor. B Perform device-assisted enteroscopy and/or obtain cross-sectional imaging, depending on local availability and expertise, to confirm the diagnosis of a subepithelial mass detected by small bowel capsule endoscopy. B Video capsule endoscopy: perform small bowel capsule endoscopy in patients with an increased risk of a small bowel tumor. B Do not obtain specific investigations before small bowel capsule endoscopy in patients with a suspected small bowel tumor unless there is a risk of capsule retention. B Biopsy: https://web.pathway.md/diseases/recaumaGTpYLUoddL 2/5 6/24/23, 3:14 PM Small bowel adenocarcinoma Pathway As per ESGE 2023 guidelines, perform biopsy sampling and tattooing of its location by device- assisted enteroscopy when there is an uncertain diagnosis of a small bowel tumor at capsule endoscopy. B As per JGCA 2022 guidelines: Consider obtaining an endoscopic diagnosis when endoscopic treatment is being considered, recognizing that histological diagnosis by biopsy is the standard for differentiating adenomas from cancers. C Consider evaluating by endoscopic gross type and coloration to differentiate intramucosal from submucosal carcinoma. C 4. Medical management Systemic therapy: As per SITC 2023 guidelines, offer pembrolizumab monotherapy in patients with previously treated, advanced high MSI/MMR-deficient small bowel adenocarcinoma. Offer dostarlimab monotherapy for MMR-deficient tumors only. B Show 2 more As per JGCA 2022 guidelines, avoid offering postoperative adjuvant therapy for the treatment of resectable small bowel cancer, including resectable duodenal cancer. D Show 2 more As per SNFGE 2018 guidelines, consider offering chemotherapy in patients with posterior invasion preventing R0 resection of cancer and involved organs and structures to make this lesion removable. E Show 7 more 5. Therapeutic procedures Endoscopic stenting: As per ESGE 2023 guidelines, consider performing enteroscopic placement of self-expanding metal stents as an alternative option to surgery for the palliation of malignant small bowel strictures. C As per ASGE 2021 guidelines, consider performing placement of self-expandable metallic stents or performing surgical gastrojejunostomy in patients with incurable malignant gastric outlet obstruction undergoing palliative intervention. Decide between these approaches based on patient characteristics and preferences, multidisciplinary input, and local expertise. C Show 2 more 6. Surgical interventions Surgical resection, indications: https://web.pathway.md/diseases/recaumaGTpYLUoddL 3/5 6/24/23, 3:14 PM Small bowel adenocarcinoma Pathway As per JSHBPS/JGCA 2022 guidelines, consider performing additional surgery after endoscopic treatment in cases of submucosal carcinoma and vascular invasion. C As per SFRO/SFED/SFCD/UNICANCER/GERCOR/FFCD/SNFGE 2018 guidelines, perform first resection in patients with no distant metastasis (M0), unless posterior invasion preventing R0 resection of the cancer and involved organs and structures is present. Consider offering preoperative treatment to make this lesion removable. E Show 4 more Surgical resection, technical considerations: As per JSHBPS/JGCA 2022 guidelines: Avoid performing procedures other than pancreatoduodenectomy in cases of duodenal cancer deeper below the submucosa. D Consider performing lymph node dissection in the surgical treatment of duodenal cancer, but consider omitting it for intramucosal lesions. C As per SFRO/SFED/SFCD/UNICANCER/GERCOR/FFCD/SNFGE 2018 guidelines, consider performing "no-touch" technique and the first ligature of the vessels. E Show 8 more Gastrojejunostomy: Consider performing surgical gastrojejunostomy or placing self-expandable metallic stents in patients with incurable malignant gastric outlet obstruction undergoing palliative intervention. Decide between these approaches based on patient characteristics and preferences, multidisciplinary input, and local expertise. C Consider preferring surgical gastrojejunostomy in patients with a life expectancy of > 6 months and good performance status. 7. Follow-up and surveillance Clinical and imaging surveillance: As per JGCA 2022 guidelines, consider obtaining careful follow-up with various imaging tests after surgical treatment of duodenal cancer in order to detect distant metastasis and local recurrence. C As per SNFGE 2018 guidelines, perform clinical examination every 3 months for 2 years and then every 6 months for 3 years after treatment. E Show 2 more Endoscopic surveillance: As per ESGE 2023 guidelines, do not perform small bowel capsule endoscopy in the follow-up of patients with treated small bowel tumors. D As per JGCA 2022 guidelines, consider obtaining endoscopic surveillance after endoscopic treatment for early detection of local recurrence and metachronous lesions. C https://web.pathway.md/diseases/recaumaGTpYLUoddL 4/5 6/24/23, 3:14 PM Small bowel adenocarcinoma Pathway References 1. Marco Pennazio, Emanuele Rondonotti, Edward J Despott et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy ESGE Guideline Update 2022. Endoscopy. 2023 Jan;55 1 58 95. Open 2. Kenji Nakagawa, Masayuki Sho, Mitsuhiro Fujishiro et al. Clinical practice guidelines for duodenal cancer 2021. J Gastroenterol. 2022 Dec;57 12 927 941. Open 3. Christophe Locher, Blaise Batumona, Pauline Afchain et al. Small bowel adenocarcinoma: French intergroup clinical practice guidelines for diagnosis, treatments and follow-up SNFGE, FFCD, GERCOR, UNICANCER, SFCD, SFED, SFRO . Dig Liver Dis. 2018 Jan;50 1 15 19. Open 4. Ronan J Kelly, Katherine Bever, Joseph Chao et al. Society for Immunotherapy of Cancer SITC clinical practice guideline on immunotherapy for the treatment of gastrointestinal cancer. J Immunother Cancer. 2023 Jun;11 6):e006658. Open 5. ASGE Standards of Practice Committee, Terry L Jue, Andrew C Storm et al. ASGE guideline on the role of endoscopy in the management of benign and malignant gastroduodenal obstruction. Gastrointest Endosc. 2021 Feb;93 2 309 322.e4. Open 6. Benson AB, Venook AP, Al-Hawary MM et al. Small Bowel Adenocarcinoma, Version 1.2020, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2019 Sep 1;17 9 1109 1133. Open 7. Benson AB, Venook AP, Al-Hawary MM et al. Small Bowel Adenocarcinoma, Version 1.2020, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw. 2019 Sep 1;17 9 1109 1133. Open 8. Jean-Marc Dumonceau, Andrea Tringali, Ioannis S Papanikolaou et al. Endoscopic biliary stenting: indications, choice of stents, and results: European Society of Gastrointestinal Endoscopy ESGE Clinical Guideline Updated October 2017. Endoscopy. 2018 Sep;50 9 910 930. Open https://web.pathway.md/diseases/recaumaGTpYLUoddL 5/5