Multiple synchronous adenocarcinomas of the small bowel in a young patient: A case report.

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Multiple synchronous adenocarcinomas of the small bowel in a young patient: A case report.

Int J Surg Case Rep. 2017 Dec 27;42:250-253

Authors: Cavalla C, Oppliger F, Schiappacasse G, Valderrama R, Castiblanco A, Margarit S

Abstract
INTRODUCTION: 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.
PRESENTATION OF CASE: 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.
DISCUSSION: 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.
CONCLUSION: 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.

PMID: 29324371 [PubMed - as supplied by publisher]

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