Abstract

A 70-year old man with a 50-year history of Crohn’s disease developed crampy lower abdominal pain, bloating, and a decreased number of bowel movements. Past medical history was remarkable for hypertension and atrial fibrillation. Abdominal examination was unremarkable. Laboratory testing was normal. Computed tomography of the abdomen showed an ileal stricture, 25 cm in length (Figure A). No other abnormalities were detected. Colonoscopy was performed to the terminal ileum where the stricture was identified. Biopsy specimens confirmed Crohn’s ileitis. Endoscopic dilatation could not be performed because of the length of the stricture and the patient was referred for ileocolonic resection. The sections of the resected small intestine showed a Crohn’s-related stricture. Additional incidental findings included a Meckel’s diverticulum containing 3.5-cm of heterotopic pancreas (Figure B), also known as a pancreatic rest, with an intraductal papillary mucinous neoplasia (IPMN) (Figure C, arrow) with high-grade dysplasia (Figure D, arrow). Subsequent abdominal magnetic resonance imaging and magnetic resonance cholangiopancreatography (Figure E) showed multiple pancreatic cysts that communicated with a normal-caliber main pancreatic duct. These features are consistent with a diagnosis of branch-duct IPMN (BD-IPMN). Heterotopic pancreas is pancreatic tissue found anywhere along the gastrointestinal tract that lacks an anatomic and vascular connection to the normal pancreas. IPMN arising from heterotopic pancreas is rare, with very few cases reported.1Tsapralis D. Charalabopoulos A. Karamitopoulou E. et al.Pancreatic intraductal papillary mucinous neoplasm with concomitant heterotopic pancreatic cystic neoplasia of the stomach: a case report and review of the literature.Diagn Pathol. 2010; 5: 4Google Scholar Furthermore, there have been only 32 reported cases of malignancy arising within the heterotopic pancreatic tissue and the true incidence is unknown.2Song J.Y. Han J.Y. Choi S.K. et al.Adenocarcinoma with intraductal papillary mucinous neoplasm arising in jejunal heterotopic pancreas.Korean J Pathol. 2012; 46: 96-100Google Scholar IPMNs first were reported in 1982 and are defined as intraductal grossly visible epithelial neoplasms of mucin-producing cells arranged in papillary formations, originating in the main pancreatic ducts or its branches.3Tanaka M. Fernandez-del Castillo C. Adsay V. et al.International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas.Pancreatology. 2012; 12: 183-197Google Scholar IPMNs can affect the main pancreatic duct (main-duct IPMN), branch ducts (BD-IPMN), or both the main and side branches (mixed-IPMN). Management consists of surveillance, or surgical resection in cysts deemed to be at high risk of malignant transformation. Surgical resection is recommended for most patients with main- or mixed-duct IPMN because high-grade or invasive adenocarcinoma is found in between 58% to 63% of patients who undergo surgical resection.3Tanaka M. Fernandez-del Castillo C. Adsay V. et al.International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas.Pancreatology. 2012; 12: 183-197Google Scholar In contrast, BD-IPMNs are associated with a much lower risk of malignant transformation, with 25% of patients who undergo surgical resection found to have high-grade or invasive carcinoma.3Tanaka M. Fernandez-del Castillo C. Adsay V. et al.International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas.Pancreatology. 2012; 12: 183-197Google Scholar To determine appropriate management, BD-IPMNs should be characterized in terms of size, presence of enhancing solid component, mural nodule, suspicious cytology, or main duct involvement. Consensus guidelines recommend surgery for symptomatic BD-IPMNs, or those with high-risk features.3Tanaka M. Fernandez-del Castillo C. Adsay V. et al.International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas.Pancreatology. 2012; 12: 183-197Google Scholar Surveillance imaging (computed tomography, magnetic resonance imaging, or endoscopic ultrasound) is recommended for the remainder of BD-IPMNs, with surveillance intervals determined by the size of the cyst.

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