There have been several reports describing the effects of CXCR4 antagonists (AMD3100, AMD11070 and KRH3955) in the treatment of malignant tumors, including breast cancer [21], small cell lung cancer [22], cholangiocarcinoma [23], gastric cancer [24] and pancreatic cancer [17, 25, 26]. Here, CXCR4 is linked to familial pancreatic carcinoma.