{"entity":{"id":"localised-small-bowel-adenocarcinoma","kind":"cancer","name":"Localised small bowel adenocarcinoma (stage I to III, resected)","aka":["Resectable small bowel adenocarcinoma","Duodenal adenocarcinoma, localised","Jejunal and ileal adenocarcinoma, localised","Stage I to III SBA"],"tldr":"Localised small bowel adenocarcinoma is cancer of the duodenum, jejunum or ileum that has not spread beyond nearby lymph nodes and can be removed by surgery, the only cure. Duodenal tumours need a Whipple operation and tumours further along a segmental resection; chemotherapy afterwards is offered for node-positive disease by analogy with colon cancer while the BALLAD trial tests whether it helps.","summary":"Small bowel adenocarcinoma is rare because the small intestine, despite making up most of the length of the gut, produces few cancers. About half arise in the duodenum, where they present with obstruction, bleeding or jaundice and are found at endoscopy, and the rest in the jejunum and ileum, where they are found late after months of obstruction or anaemia; capsule endoscopy, CT enterography and double-balloon enteroscopy have shortened that delay. Predisposing conditions matter: Crohn's disease causes ileal tumours, coeliac disease jejunal ones, familial adenomatous polyposis duodenal and ampullary ones, and Lynch syndrome tumours anywhere, so germline testing and mismatch repair testing are recommended for all patients. Mismatch repair deficiency is found in a larger share than in colon cancer, and HER2 amplification and KRAS mutations in others.\n\nSurgery follows the site: pancreaticoduodenectomy (Whipple procedure) for tumours of the first and second parts of the duodenum, segmental resection with wide lymphadenectomy for the distal duodenum, jejunum and ileum, and right hemicolectomy for terminal ileal tumours, with at least eight nodes examined for accurate staging. Node involvement is the main prognostic factor. No randomised trial had ever tested adjuvant chemotherapy until the international BALLAD trial, which randomised patients with resected stage I to III disease to observation or to fluoropyrimidine chemotherapy with or without oxaliplatin; pending its final results, the NCCN guideline recommends adjuvant CAPOX or FOLFOX for stage III and high-risk stage II disease on the colon cancer model, and considers observation for stage I and low-risk stage II tumours. Mismatch-repair-deficient tumours may gain less from fluoropyrimidines, and neoadjuvant or adjuvant checkpoint inhibition for them is under study. Circulating tumour DNA is being tested to identify patients with residual disease after surgery.","asOf":"2026-09-18","wikipedia":"https://en.wikipedia.org/wiki/Small_intestine_cancer","links":[{"label":"NCCN Small Bowel Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1490"},{"label":"BALLAD (NCT02502370)","url":"https://clinicaltrials.gov/study/NCT02502370"},{"label":"Wikipedia","url":"https://en.wikipedia.org/wiki/Small_intestine_cancer"}],"tags":["subtype-page","gastrointestinal"],"related":["advanced-small-bowel-adenocarcinoma","small-bowel","small-intestinal-net","ampullary"],"cancers":[],"sections":[],"technologies":["endoscopy","ct","germline-testing","cytotoxic-chemotherapy","mri"],"targets":["kras","her2"],"drugs":["capox","folfox"],"companies":[],"institutions":[],"pathways":[],"terms":["whipple","lymphadenectomy","msi","lynch-syndrome","hereditary-cancer-syndromes","colectomy"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":["paper-overman-capox-small-bowel-adenocarcinoma-jco-2009","paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020"],"journals":[],"dependsOn":[],"notes":[],"group":"gastrointestinal","burden":"About half of small bowel adenocarcinomas are found before distant spread, most often in the duodenum; many are linked to Crohn's disease, coeliac disease, Lynch syndrome or familial adenomatous polyposis.","subtypes":["Localised duodenal adenocarcinoma (Whipple procedure; FAP-associated in some)","Localised jejunal adenocarcinoma (coeliac-associated in some)","Localised ileal adenocarcinoma (Crohn's-associated in some)","Stage I to low-risk stage II small bowel adenocarcinoma (observation after surgery)","Stage III or high-risk stage II small bowel adenocarcinoma (adjuvant CAPOX or FOLFOX)","Mismatch-repair-deficient localised small bowel adenocarcinoma (Lynch syndrome screening)"],"biomarkers":["Mismatch repair and microsatellite status (all patients)","Node status and number of nodes examined (at least eight)","Germline testing for Lynch syndrome, FAP and Peutz-Jeghers where indicated","HER2 amplification and KRAS status (baseline for later therapy)","Circulating tumour DNA after surgery (investigational)","Crohn's disease or coeliac disease history"],"standardOfCare":[{"setting":"Diagnosis and staging","approach":"Endoscopy or enteroscopy with biopsy, CT of chest, abdomen and pelvis, mismatch repair testing and germline assessment.","refs":["endoscopy","ct","msi","germline-testing","lynch-syndrome"],"guideline":{"version":"NCCN Guidelines: Small Bowel Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1490"}},{"setting":"Duodenal tumours","approach":"Pancreaticoduodenectomy for proximal duodenal tumours; segmental resection for distal duodenal tumours; endoscopic resection only for adenomas.","refs":["whipple","lymphadenectomy"],"guideline":{"version":"NCCN Guidelines: Small Bowel Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1490"}},{"setting":"Jejunal and ileal tumours","approach":"Segmental resection with wide mesenteric lymphadenectomy; right hemicolectomy for terminal ileal tumours.","refs":["lymphadenectomy","colectomy"],"guideline":{"version":"NCCN Guidelines: Small Bowel Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1490"}},{"setting":"After surgery","approach":"Observation for stage I and low-risk stage II; adjuvant CAPOX or FOLFOX for stage III and high-risk stage II, extrapolated from colon cancer pending BALLAD.","refs":["capox","folfox","cytotoxic-chemotherapy"],"guideline":{"version":"NCCN Guidelines: Small Bowel Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1490"}},{"setting":"Surveillance","approach":"CT and CEA every six to twelve months; endoscopic surveillance of the remaining duodenum in FAP.","refs":["ct","endoscopy"],"guideline":{"version":"NCCN Guidelines: Small Bowel Adenocarcinoma","url":"https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1490"}}],"stateOfArt":["Surgery cures a good share of patients with node-negative disease.","BALLAD is the first randomised adjuvant trial in the disease.","Universal mismatch repair testing finds Lynch syndrome families and identifies candidates for immunotherapy."],"history":[{"year":1935,"title":"Whipple describes pancreaticoduodenectomy","refs":["whipple"]},{"year":2014,"title":"Retrospective series suggest a benefit from adjuvant chemotherapy in node-positive disease","refs":["capox","folfox"]},{"year":2015,"title":"BALLAD opens as an international trial of adjuvant chemotherapy versus observation","refs":[]},{"year":2019,"title":"NCCN publishes its first guideline for small bowel adenocarcinoma","refs":[]}],"pipeline":["capox","folfox","signatera"],"openProblems":["Whether adjuvant chemotherapy improves survival awaits the full BALLAD results.","Diagnosis is often delayed by months because the small bowel is hard to image.","The optimal extent of lymphadenectomy is undefined.","Patients with Crohn's disease are hard to screen."],"parent":"small-bowel"},"route":"/cancers/localised-small-bowel-adenocarcinoma/","neighbours":{"cancer":[{"id":"advanced-small-bowel-adenocarcinoma","kind":"cancer","name":"Advanced and metastatic small bowel adenocarcinoma","route":"/cancers/advanced-small-bowel-adenocarcinoma/"},{"id":"ampullary","kind":"cancer","name":"Ampullary cancer (ampulla of Vater)","route":"/cancers/ampullary/"},{"id":"small-intestinal-net","kind":"cancer","name":"Small intestinal neuroendocrine tumours","route":"/cancers/small-intestinal-net/"},{"id":"small-bowel","kind":"cancer","name":"Small intestine cancer (small bowel adenocarcinoma)","route":"/cancers/small-bowel/"}],"term":[{"id":"colectomy","kind":"term","name":"Colectomy","route":"/terms/colectomy/"},{"id":"endoscopy","kind":"term","name":"Endoscopy (EGD, EUS, ERCP)","route":"/terms/endoscopy/"},{"id":"hereditary-cancer-syndromes","kind":"term","name":"Hereditary cancer syndromes","route":"/terms/hereditary-cancer-syndromes/"},{"id":"lymphadenectomy","kind":"term","name":"Lymphadenectomy (lymph node dissection)","route":"/terms/lymphadenectomy/"},{"id":"lynch-syndrome","kind":"term","name":"Lynch syndrome","route":"/terms/lynch-syndrome/"},{"id":"msi","kind":"term","name":"Microsatellite instability (MSI-H) / mismatch repair deficiency (dMMR)","route":"/terms/msi/"},{"id":"whipple","kind":"term","name":"Whipple procedure (pancreaticoduodenectomy)","route":"/terms/whipple/"}],"technology":[{"id":"ct","kind":"technology","name":"CT (computed tomography)","route":"/technologies/ct/"},{"id":"cytotoxic-chemotherapy","kind":"technology","name":"Cytotoxic chemotherapy","route":"/technologies/cytotoxic-chemotherapy/"},{"id":"germline-testing","kind":"technology","name":"Germline (hereditary) testing","route":"/technologies/germline-testing/"},{"id":"mri","kind":"technology","name":"MRI","route":"/technologies/mri/"}],"target":[{"id":"her2","kind":"target","name":"HER2","route":"/targets/her2/"},{"id":"kras","kind":"target","name":"KRAS","route":"/targets/kras/"}],"drug":[{"id":"capox","kind":"drug","name":"CAPOX (capecitabine, oxaliplatin)","route":"/drugs/capox/"},{"id":"folfox","kind":"drug","name":"FOLFOX (5-FU, leucovorin, oxaliplatin)","route":"/drugs/folfox/"},{"id":"signatera","kind":"drug","name":"Signatera","route":"/drugs/signatera/"}],"paper":[{"id":"paper-overman-capox-small-bowel-adenocarcinoma-jco-2009","kind":"paper","name":"Phase 2 study of capecitabine and oxaliplatin for advanced adenocarcinoma of the small bowel and ampulla of Vater","route":"/key-papers/paper-overman-capox-small-bowel-adenocarcinoma-jco-2009/"},{"id":"paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020","kind":"paper","name":"The 2019 WHO classification of tumours of the digestive system","route":"/key-papers/paper-who-2019-digestive-system-tumours-nagtegaal-histopathology-2020/"}]}}