Neoplasia mucinosa papilar intraductal com fistulização gástrica e duodenal: uma forma rara de apresentação

Intraductal papillary mucinous neoplasm (IPMN) is a rare pancreatic cystic neoplasm with high risk of malignancy. We present the case of a 64-year-old male with steatorrhea and weight loss (17% total body weight). Colonoscopy detected presence of oil droplets in stools. Esophagogastroduodenoscopy revealed a fistulous orifice with mucous drainage in the duodenal bulb and a bulging ampulla of Vater, possibly by external compression; Abdominal CT showed the presence of a cystic lesion in the pancreatic head, 5.8 cm in diameter, significant dilation of wirsung and slight dilation of common bile duct. EUS revealed a multicystic lesion with irregular contours in the pancreatic head, suggestive of IPMN. EUS guided fine needle citologic examination confirmed IPMN. Abdominal MRI detected fistulization to the duodenum and stomach. Pancreatoduodenectomy was performed and a pathological diagnosis of mucinous adenocarcinoma in IPMN was obtained. There has been no evidence of recurrence during 14 months of follow-up. Most patients diagnosed with IPMN are assymptomatic, steatorrhea is the less frequent form of presentation. Fistulization to adjacent organs occurs in 7-15% cases, combined fistula to duodenum and stomach in only 6%.