Molecular studies suggest that its carcinogenic pathways involve abnormal activation of the ErbB2/ErbB3-MUC4 axis, activation of the PI3K and MAPK pathways, and frequent mutations in classical pancreatic cancer driver genes such as KRAS; however, the lack of specific molecular biomarkers and typical imaging findings makes clinical diagnosis challenging. The gene discussed is ERBB3; the disease is familial pancreatic carcinoma.