Dorsal pancreas agenesis and ductal adenocarcinoma: surgical implications of an extremely rare association.

It is presented the case of a 44-year-old woman with ductal adenocarcinoma of the pancreatic head with associated dorsal pancreas agenesis. In this case, curative intent surgery implies removal of the whole pancreas with its consequences--parental insulin requirement and exocrine pancreatic insufficiency. A review of the literature on the previously reported cases was performed.