Molecular Characterization and Pathogenesis of Intraductal Papillary Mucinous Neoplasms of the Pancreas

2015 ◽  
Vol 55 (4) ◽  
pp. 352-363 ◽  
Author(s):  
Julia Benzel ◽  
Volker Fendrich

Intraductal papillary mucinous neoplasms (IPMNs) of the pancreas are noninvasive neoplasms which occur in the main pancreatic duct or its major branches. IPMNs have an important meaning in the clinic and in research since they represent around 20% of all resected pancreatic neoplasms. Morphologically, branch duct, main duct and mixed-type IPMNs can be distinguished. Histologically, they can be divided into gastric, intestinal, pancreatobiliary and oncocytic type. There are different mutations in genes such as KRAS, GNAS, RNF43 and p53. The expression of miRNAs is specific to IPMNs; altogether, 14 miRNAs have been identified so far which are differently expressed in all IPMNs in contrast to normal pancreatic tissue. It has also been observed that methylation levels can be altered in IPMNs. This review summarizes the molecular characteristics of IPMNs of the pancreas and presents currently known markers.

Surgery ◽  
2014 ◽  
Vol 156 (3) ◽  
pp. 611-621 ◽  
Author(s):  
Klaus Sahora ◽  
Carlos Fernández-del Castillo ◽  
Fei Dong ◽  
Giovanni Marchegiani ◽  
Sarah P. Thayer ◽  
...  

Author(s):  
F. Gallucci ◽  
D. Avolio ◽  
R. De Ritis ◽  
L. Ferrara ◽  
U. Valentino ◽  
...  

Intraductal papillary mucinous neoplasms (IPMNs) are rare pancreatic tumors, accounting for less than 1-2% of all neoplasms of the pancreas. The main characteristic of IPMNs is their favorable prognosis, as these pre-malignant or malignant lesions are usually slow-growing tumors and radical surgery is frequently possible. According to the localization of the lesions, three different tumor types have been identified: the main-duct IPMN, the branch-duct IPMN and the mixed-type IPMN (involving both the main pancreatic duct and the side branches). IMPNs do not present pathognomonic signs or symptoms. The obstruction of the main pancreatic duct system may cause abdominal pain and acute pancreatitis (single or recurrent episodes). The tumor may be incidentally discovered in asymptomatic patients, particularly in those with branch-duct IPMNs. In clinical practice, any non-inflammatory cystic lesion of the pancreas should be considered as possible IPMN. Computed tomography, magnetic resonance imaging with cholangiopancreatography and endoscopic ultrasonography can localize an IPMN and assess its morphology and size. The choice between non-operative and surgical management depends on the risk of malignancy and on the definitive distinction between benign and malignant IPMNs. Main-duct IPMNs have a high risk of malignant degeneration, especially in older patients. The clinical and radiological features, as well as treatment and outcome, of eight patients with IPMN (five with main-duct, two with branch-duct and one with mixed-type) observed by the authors over the last ten years are presented.


2014 ◽  
Vol 2014 ◽  
pp. 1-9 ◽  
Author(s):  
Stefano Palmucci ◽  
Claudia Trombatore ◽  
Pietro Valerio Foti ◽  
Letizia Antonella Mauro ◽  
Pietro Milone ◽  
...  

Intraductal papillary mucinous neoplasms (IPMNs) represent a group of cystic pancreatic neoplasms with large range of clinical behaviours, ranging from low-grade dysplasia or borderline lesions to invasive carcinomas. They can be grouped into lesions originating from the main pancreatic duct, main duct IPMNs (MD-IPMNs), and lesions which arise from secondary branches of parenchyma, denominated branch-duct IPMNs (BD-IPMNs). Management of these cystic lesions is essentially based on clinical and radiological features. The latter have been very well described in the last fifteen years, with many studies published in literature showing the main radiological features of IPMNs. Currently, the goal of imaging modalities is to identify “high-risk stigmata” or “worrisome feature” in the evaluation of pancreatic cysts. Marked dilatation of the main duct (>1 cm), large size (3–5 cm), and intramural nodules have been associated with increased risk of degeneration. BD-IPMNs could be observed as microcystic or macrocystic in appearance, with or without communication with main duct. Their imaging features are frequently overlapped with cystic neoplasms. The risk of progression for secondary IPMNs is lower, and subsequently an imaging based follow-up is very often proposed for these lesions.


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