Letter to the Editor Carcinoid of the Minor Duodenal Papilla Associated with Pancreas Divisum: Case Report and Review of the Literature

Carcinoid tumors of Vater’s ampulla are the most common among the extremely rare primary ampullary neuroendocrine tumors, accounting for less than 0.35% of all gastrointestinal carcinoids and are frequently associated with Von Recklinghausen’s disease. Carcinoid tumors of Vater’s ampulla are difficult to diagnose preoperatively because of their relatively small size and submucosal location. Consequently, their true nature is usually only discovered after appropriate immunocytochemical, histochemical, or ultrastructural studies. Carcinoid tumors of the minor papilla are also very rare. To the best of our knowledge, only 7 cases have been reported in the literature. That carcinoid tumors of the major duodenal papilla have been described more frequently than those of the minor papilla is probably because those of the major duodenal papilla often give rise to symptoms (for example jaundice and pain) that demand intensive examination or surgery; while those of the minor papilla are frequently asymptomatic. These gastrointestinal tumors appear to be hormonally inactive, and abdominal pain is the main clinical feature. Pancreas divisum is the most common congenital variant of the pancreatic anatomy and occurs when the ductal systems of the ventral and dorsal pancreatic ducts fail to fuse. Because the ducts are not completely united, most of the pancreatic exocrine secretions enter the duodenum via the dorsal duct and minor papilla. Of the 7 cases of carcinoid tumors of the minor papilla reported in the literature, 4 were associated with pancreas divisum. This report describes a case of an endocrine tumor of the carcinoid type, immunohistochemically a somatostatinoma, which was localized in the minor papilla and associated with pancreas divisum. CASE REPORT