Agenesis of the Dorsal Pancreas and Its Possible Link to Pancreatic Neuroendocrine Tumors: Case Insights.

Abstract

BACKGROUND Agenesis of the dorsal pancreas (ADP) is a rare congenital anomaly resulting from incomplete development of the dorsal pancreatic bud, affecting the body and tail of the pancreas. While often asymptomatic, it can present with clinical abnormalities such as abdominal pain, diabetes, or obstructive jaundice, and may be associated with chronic pancreatitis and tumors. We report the third documented case of a high-grade neuroendocrine tumor located at the head of the pancreas in a patient with complete ADP, along with a review of the literature. CASE REPORT An 86-year-old man presented with jaundice caused by a 3-cm pancreatic mass compressing the bile duct, confirmed through imaging to be associated with ADP. Surgical intervention was declined, and a combined laparoscopic and radiological approach was used to place a biliary stent, alleviating symptoms. Histopathological analysis confirmed a poorly differentiated neuroendocrine carcinoma (NEC). Despite initial symptomatic relief, the patient died due to complications 3 months later. CONCLUSIONS This report underscores the diagnostic challenges of ADP and its potential association with pancreatic neoplasms. Improved imaging techniques such as computed tomography (CT), magnetic resonance imaging (MRI), and endoscopic ultrasound (EUS) facilitate diagnosis and differentiation from similar conditions. The association between ADP and pancreatic tumors should warn us to be watchful with every patient diagnosed with ADP and focus on the early detection of possible malignancies. Further research is needed to clarify the genetic and developmental mechanisms linking ADP to tumorigenesis and to explore targeted therapeutic approaches.