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Atypical Presentation of Main-Duct Intraductal Papillary Mucinous Neoplasm

Context Intraductal papillary mucinous neoplasm is a ductal epithelial tumor characterized by dilation of the pancreatic duct due to mucus production and is a pre-neoplastic disease. The most used diagnostic studies are computed tomography scan and magnetic resonance cholangio-pancreatography. Case Report A Sixty-three-year-old female presented with fever and a painful palpable abdominal mass in the left flank. Ultrasonography revealed a supra-aponeurotic fluid collection that was punctured, obtaining purulent material. Computed tomography scan showed a supra aponeurotic collection communicated with a cystic image that extended behind the stomach and involved the pancreas, which also showed dilatation of the Wirsung duct. Magnetic resonance cholangio-pancreatography showed dilatation of the main pancreatic duct from the pancreatic head up to the tail of the pancreas and was also communicated with a large cystic cavity behind the gastric antrum. The intraoperative finding evidenced a communication of the supra-aponeurotic collection with a cystic tumor that involved the pancreas and the posterior surface of the stomach. Left pancreatectomy with splenectomy and antrectomy was performed, sectioning the pancreas at the neck. Frozen section of the pancreatic duct surface revealed severe dysplasia at the main duct. Resection was completed by performing pancreatoduodenectomy. The pathologic analysis revealed cystic papillary mucinous neoplasia with foci of high-grade dysplasia and invasive colloid-type carcinoma. Conclusions This is an atypical presentation of type 1 intraductal papillary mucinous neoplasm, but the diagnosis was suspected in the preoperative period with the computed tomography scan, the magnetic resonance cholangio-pancreatography and the upper GI endoscopy. It is important to know all the possible differential diagnosis to decide the best surgical procedure. In cases of invasive type 1 intraductal papillary mucinous neoplasm, involving other organs, an aggressive surgical resection is the best choice for the patient.


Gustavo Kohan, Ornella A Ditulio, Gabriel Raffin, Alejandro Faerberg, Fernando Duek

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