Connective tissue growth factor (CTGF), a member of the CCN family of secreted proteins, is involved in ECM production, desmoplasia, and tumor progression.380 Preclinical data indicated that CTGF antagonism with the therapeutic monoclonal human antibody pamrevlumab enhanced the response to gemcitabine in pancreatic cancer mouse models.381 The safety and efficacy of the agent in combination with gemcitabine and nab-paclitaxel for the treatment of patients with unresectable pancreatic cancer were evaluated in a phase 1/2 trial (NCT02210559). Here, CCN2 is linked to familial pancreatic carcinoma.