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Primary pancreatic cystic neoplasms are being recognized with increasing frequency due to modern imaging techniques. In addition to the more common cystic neoplasms-serous cystadenoma, primary mucinous cystic neoplasm, and intraductal papillary mucinous neoplasm-there are many other less common neoplasms that appear as cystic lesions. These cystic neoplasms include solid pseudopapillary neoplasm of the pancreas (the most common rare cystic neoplasm), cystic neuroendocrine neoplasm, cystic degeneration of otherwise solid neoplasms, and then the exceedingly rare cystic acinar cell neoplasm, intraductal tubular neoplasm, angiomatous neoplasm, lymphoepithelial cysts (not true neoplasms), and few others of mesenchymal origin. While quite rare, the pancreatic surgeon should at the least consider these unusual neoplasms in the differential diagnosis of potentially benign or malignant cystic lesions of the pancreas. Moreover, each of these unusual neoplasms has their own natural history/tumor biology and may require a different level of operative aggressiveness to obtain the optimal outcome., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Intraductal papillary mucinous neoplasms (IPMNs) represent about 25% of all primary pancreatic cystic neoplasms and are increasingly recognized during the last two decades. They are characterized by intraductal proliferation of neoplastic mucinous cells forming papillary projections into the pancreatic ductal system, which is typically dilated and contains globules of mucus. IPMNs may be multifocal and have malignant potential. Modern imaging is essential in establishing preoperative diagnosis and in differentiating different subtypes of IPMNs (i.e., main-duct vs. branch-type disease). Endoscopic retrograde or magnetic resonance cholangiopancreatography accurately delineate the morphologic changes of the pancreatic ductal system. Endoscopic ultrasonography (usually used in conjunction with image-guided FNA and analysis of the aspirated material) is commonly used for differential diagnosis of IPMNs from other pancreatic cystic lesions. Surgical resection (usually anatomic pancreatectomy, depending on the location of the disease) is the treatment of choice. Total pancreatectomy may occasionally be required in selected patients, but is associated with formidable long-term morbidity. A conservative approach has recently been proposed for carefully selected patients with branch-duct IPMNs. Recurrences following surgical resection can be observed, especially in patients with multifocal disease or in the presence of underlying malignancy., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Primary pancreatic cystic neoplasms have been recognized increasingly during the two recent decades and include mainly serous cystic neoplasms, mucinous cystic neoplasms, and intraductal papillary mucinous neoplasms. Serous cystic neoplasms represent about 30% of all cystic neoplasms of the pancreas and are characterized by their microcystic appearance (on imaging, macroscopically, and microscopically) and their benign biologic behavior. Modern diagnostic methodology allows the preoperative diagnosis with an acceptable accuracy. Currently, indications for resection of serous cystic neoplasms of the pancreas include the presence of symptoms, size > 4 cm (because these 'large' neoplasms have a more rapid growth rate and probably will soon become symptomatic), and any uncertainty about the diagnosis of a serous versus a mucinous cystic neoplasm. Resection should also be considered for lesions in the body/tail of the pancreas. Conservative treatment is a reasonable option in selected patients (for example in the presence of small, asymptomatic lesions in the pancreatic head, especially in the frail or elderly patient)., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

Mucinous cystic neoplasms (MCNs) of the pancreas represent one of the most common primary pancreatic cystic neoplasms, accounting for approximately half of these cases. MCNs are observed almost exclusively in women, and most commonly are located in the body/tail of the pancreas. In contrast to SCNs, MCNs have malignant potential. Proliferative changes (hyperplasia with or without atypia, borderline changes, non-invasive or carcinomas in-situ, and invasive carcinomas) can often be observed within the same neoplasm. Several risk factors for the presence of underlying malignancy within an MCN have recently been recognized. Cross-sectional imaging is of key importance for the diagnostic evaluation of patients with a cystic pancreatic lesion. Cyst fluid examination (cytology, biochemical/genetic analysis) is possible by using fine needle aspiration of the MCN, usually under endoscopic guidance, and may provide useful information for the differential diagnosis. Since MCNs have malignant potential, surgical resection is the treatment of choice., (Copyright © 2010 Elsevier Ltd. All rights reserved.)