Hôpital Saint-Louis, AP-HP, Service de gastroentérologie et cancérologie digestive, Université de Paris-Cité, 1 avenue Claude Vellefaux, 75010 Paris
Hôpital Saint-Louis, AP-HP Service de radiologie, Université de Paris-Cité, Paris
Hôpital Georges Pompidou, AP-HP, Service de biologie, Université de Paris-Cité, Paris
Hôpital Saint-Antoine, AP-HP, Service d’anatomo-pathologie, Sorbonne Université, Paris
Correspondance : T. Aparicio
Small bowel adenocarcinomas (SBA) are rare tumors, but their incidence is increasing. The commonest primary location is the duodenum. Even though SBA are more often sporadic, some diseases are risk factors, such as Crohn’s disease and some genetic predispositions to cancer as Lynch syndrome or familial adenomatous polyposis. Molecular phenotyping revealed some differences between SBA and colorectal cancer. Early diagnosis of small bowel adenocarcinoma remains difficult, despite significant radiological and endoscopic progresses. After R0 surgical resection, the main prognostic factor is lymph node invasion. An international randomized trial (BALLAD study) is ongoing to evaluate the benefit of adjuvant chemotherapy. For metastatic disease, retrospective studies suggest that platinum-based chemotherapy is the most effective treatment, but no significant improvement has been achieved since decades. A dramatic effect has been reported with immunotherapy in tumor with deficient mismatch repair system. Phase II studies are ongoing to evaluate targeted therapy in metastatic SBA. Other targeted therapies may be efficient in case of specific molecular alterations.