Small bowel adenocarcinoma (SBA) is a very rare tumor entity but occurs in up to 5% of patients suffering from familiar adenomatous polyposis (FAP). Because of nonspecific symptoms, diagnosis is usually made with delay, which contributes to high rates of metastatic disease at the time of diagnosis. The overall prognosis of SBA is poor with 5-year survival rates of 15-35%. For localized disease, complete surgical resection is the treatment of choice, whereas systemic chemotherapy is deemed indicated in tumors with metastatic spread. The optimal regimen has not been defined as yet. In October 2001, a 51-year-old woman with attenuated FAP, that had total proctocolectomy in 1994 was diagnosed with a jejunal adenocarcinoma. She subsequently underwent small bowel resection. Because a computed tomography (CT) scan in April 2002 revealed multiple liver metastases, chemotherapy with nine cycles FOLFOX6 was initiated. Afterwards, a small residual lesion in segment VIII was seen in CT scan but could not be identified by PET and at laparotomy in November 2002. In December 2003, again, a lesion was detected in S VIII. This solitary residual liver metastasis was resected in January 2004. Postoperatively, the patient received adjuvant chemotherapy with three cycles (with six applications in each cycle) 5-fluorouracil/folinic acid/irinotecan according to the AIO protocol. To date, more than 3 years after liver resection, the patient is still in complete remission and undergoes regular restaging investigations. Resection of liver metastases from SBA combined with neoadjuvant and adjuvant chemotherapy can result in extended disease-free survival and should undergo further investigation.
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