CXCR4 blocking strategies using antibodies [106] or small inhibitors [107,108] have proven to be effective to reduce metastatic burdens and a phase IIb clinical trial for a CXCR4 antagonist in combination with anti-PD1 therapy in advanced pancreatic cancer is ongoing (NCT02907099). Here, CXCR4 is linked to familial pancreatic carcinoma.