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.