Summary

Pancreatic dorsal agenesis (PDA) is a rare congenital anomaly, usually asymptomatic, that can present with abdominal pain, pancreatitis, diabetes mellitus and jaundice. Pancreatic tumours in PDA background are extremely rare, and when they occur are mainly pancreatic ductal adenocarcinoma. We present a case of a 48-year-old female patient with incidental detection of a 26×20 mm nodular lesion of the cephalic pancreas on ultrasound. Surgery was performed and gross examination revealed PDA with a tumour developed around the Wirsung duct. Histology showed a neuroendocrine tumour G1 with neural and vascular invasion. Two and half years later, the patient is alive and without tumour relapse. Awareness of the association of PDA and pancreatic tumours is fundamental in order to develop personalised follow-up programmes.