A Rare Case of IPMN Arising from Heterotopic Pancreatic Tissue and Gastroenteric Congenital Anomalies

Michaela Cellina,Giulia Van Der Byl,Chiara Floridi,Giancarlo Oliva,Marcello Alessandro Orsi

doi:10.23937/2572-3235/1510028

Abstract

We report a rare case of double Intraductal Papillary Mucinous Neoplasm with multiple foci of adenocarcinoma originating from duodenal and gastric heterotopic pancreatic tissue, associated with gastric duplication and intestinal malrotation.

Highlights

Pancreatic ectopia is defined as pancreatic tissue that lacks anatomical and vascular connection to the normal pancreas, found in 0.5% to 13% of autopsies, most frequently in duodenum, stomach and jejunum
Most visualized in portal venous phase, with a microcystic pattern [2]. These imaging aspects can consisted both with a branch duct type Intraductal Papillary Mucinous Neoplasm (IPMN) with microcystic pattern, and with a serous cystadenoma
Serous cystadenoma represents approximately 20% of cystic lesions of the pancreas, occurring in females in about 75% of cases [9], the typical imaging appearance is the microcystic form consisting of a cluster of several small cysts, each less than 1 cm in diameter, often in a honeycomb configuration, separated by fibrous septa [5]; sunburst calcifications of the central scar may be present in about 30% of cases [10]

Summary

Introduction

Pancreatic ectopia is defined as pancreatic tissue that lacks anatomical and vascular connection to the normal pancreas, found in 0.5% to 13% of autopsies, most frequently in duodenum, stomach and jejunum. The presence of heterotopic pancreas is usually asymptomatic and often discovered incidentally, pathological changes that occur within the native pancreas may occur rarely in the ectopic gland, including pancreatitis, benign and malignant lesions [1]. Intraductal Papillary Mucinous Neoplasm (IPMN) is a clinicopathological entity originating from the mucinous epithelium of the pancreatic ductal system, characterized by intraductal papillary proliferations and excessive mucin production, resulting in progressive cystic dilation of the pancreatic ducts [2]. IPMNs account for approximately of 20% of cystic pancreatic lesions and are classified in three types, on the basis of the site of the tumor involvement: Main duct IPMN, branch duct IPMN, and combined type. IPMNs can be divided into three groups: Benign without dysplasia, borderline neoplasms and carcinoma (invasive or non-invasive) [3]. Carcinoma is found in about 70% of IPMNs involving the main duct and in about 25% of IPMNs involving only side-branches [3]

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Conclusion