Adenocarcinoma of the small bowel is a rare neoplasm presented usually in elder patients as a single tumor. Its presentation as multiple tumors and in young patients is exceptional and there aren't any guidelines to orient its therapy.

We present the rare case of a sixteen-year-old woman that presents to the emergency department with an intussusception due to a small bowel tumor. The resected specimen showed multiple adenocarcinomas. A complete endoscopic and PET-CT study showed other 5 lesions from the duodenum to the ileum that were resected. Genetic counseling showed no pathogenic changes. The final staging was T2N0M0 and only surveillance was indicated. The patient is now 3 years without any recurrence.

Multiple adenocarcinomas of the small bowel are a very infrequent presentation of the disease. Most common risk factors include Crohn disease and adenomas. Its presentation is usually vague with a delay in its diagnosis. The treatment remains mainly surgical with limited use of adjuvant therapy. The most important prognostic factor is lymph node involvement with 5-year survival that can range from 3%-60% depending on the stage.

This case represents an exceptional presentation of a very rare pathology with few cases described in the literature. There isn't one single best study to stage the patient and surgery is still the standard of treatment while adjuvant therapies studies are being conducted. The young age and lack of predisposing factors or mutations leaves an open field for investigation.

Patients affected with Crohn's disease (CD) have a recognized, but low relative risk of developing small-bowel adenocarcinoma (SBA). In fact, SBA develops in 2.2% of patients who have long-standing CD and it is seldom diagnosed preoperatively because of its rarity. A retrospective analysis of all cases of SBA in CD patients since 1980 was carried out in Rouen University Hospital. Three patients with known or unknown CD who presented with SBA with long-term follow-up were analysed. In our first case, the occlusive syndrome revealed SBA and CD simultaneously. Most ileal carcinomas in CD are located in strictures and are often incidentally diagnosed postoperatively, as in our three cases. Digestive surgeons and gastroenterologists must be aware that the diagnosis of SBA in CD is often made fortuitously on histological examination after surgical resection for an occlusive syndrome. Failure to detect SBA in patients with CD results in late diagnosis, with poor survival.