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Mentions: On the CT scan, the pancreatic duct was diffusely dilated without a definite mass and the common bile duct was slightly dilated (Figure 1). Magnetic resonance cholagiopancreatography (MRCP) showed a dilated dorsal pancreatic duct crossing over the common bile duct with stenosis at the minor papailla. The ventral duct appeared to be normal and there was no communication between the dorsal and ventral ducts, indicating pancreas divisum (Figure 2). The common bile duct was slightly dilated, and narrowing of the distal common bile duct (CBD) was noted without passage disturbance. A duodenoscopic examination revealed a 1 cm yellow, bulging, nodular lesion with an intact mucosal surface in the minor papilla. The orifice of the minor papilla was not visible due to the nodule and cannulation was not possible (Figure 3A). On endoscopic retrograde cholangiopancreatography (ERCP), the major papilla appeared to be normal. Injection of contrast through the major papilla revealed a short and slender ventral duct confined to the pancreas head with no visualization of ducts in the body and tail. The ventral duct gradually diminished in caliber and was arborizing (Figure 3B). Endoscopic ultrasonography (EUS) showed a 1.3 cm ill-defined hypoechoic mass in the submucosal layer of the minor papilla (Figure 4). Multiple deep biopsies of this nodule revealed a carcinoid tumor.
Carcinoid tumor of the minor papilla in complete pancreas divisum presenting as recurrent abdominal pain
Bottom Line: She complained of intermittent epigastric pain for 6 months.A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct.The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes.
Affiliation: Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, South Korea.
Background: Tumors of the minor papilla of the duodenum are extremely rare, and they are mostly neuroendocrine tumors, such as somatostatinomas and carcinoid tumors. However, true incidence of carcinoid tumors in minor papilla might be much higher, because patients with minor papillary tumors usually remain asymptomatic. We report a very unusual case of carcinoid tumor in a patient with complete pancreas divisum with a review of the literature.
Case presentation: A 56-year-old female patient was referred for evaluation of pancreatic duct dilatation noted on abdominal ultrasonography and computerized tomography. She complained of intermittent epigastric pain for 6 months. A MRCP and ERCP revealed complete pancreas divisum with dilatation of the main pancreatic duct. On duodenoscopy, a small, yellows, subepithelial nodule was visualized at the minor papilla; biopsy of this lesion revealed a carcinoid tumor. She underwent a pylorus-preserving pancreaticoduodenectomy. The histologic evaluation showed a single nodule, 1 cm in diameter, in the submucosa with duodenal and vascular invasion and metastasis to the regional lymph nodes.
Conclusion: Although the size of the carcinoid tumor was small and the tumor was hormonally inactive, the concomitant pancreas divisum led to an early diagnosis, the tumor had aggressive behavior. Carcinoid tumors of the minor papilla should be included in the differential diagnosis of recurrent abdominal pain or pancreatitis of unknown cause.
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