How do you treat metastatic adenocarcinoma of the ampulla of Vater?

True adenocarcinoma of the ampulla of Vater is an extremely rare cancer of the GI tract (<5 pts per million). Consequently, data is mostly derived from retrospective studies or large databases with few clinical trials available, especially with locally advanced and metastatic disease.

I personally h...

Given its rarity, ampullary adenocarcinoma has often been grouped with other tumor types, such as biliary tract and small bowel cancers in prospective clinical studies (Valle et al., NEJM 2010; Overman et al., JCO 2009). As such, the optimal systemic treatment regimen has been debated and there are ...

In the resected setting: it would be prudent to ask the surgeon and pathologist about ampullary vs peri-ampullary cancer. Prognosis and treatment are different for both entities and also for subtype. As discussed above, there is not much data. Most oncologists tend to use mFOLFIRINOX or pancreatic t...

I would think the treatments of both are becoming progressively similar; mFOLFIRINOX and FOLFOXIRI are virtually the same drugs and have proven the most active in the 2 individual types.

I would not favor a gemcitabine approach: there is activity, but more desirable outcomes can be reached with alte...