Conclusion
MANEC is very rare, aggressive, rapidly spreading disease, usually with poor prognosis. (5). Although a rare cancer, MANEC presents a significant therapeutic challenge because there are no clear guidelines based on randomized trials. In our case, the patient responded well to the third line of chemotherapy, FOLFIRI3 regimen plus Sandostatin LAR . It is of great importance the early recognition of tumor rarities such as MANEC, the multidisciplinary approach from the beginning of treatment as well as the need to form a single reference center for rare tumors.