Pancreatic cystic neoplasms: a review of preoperative diagnosis and management

Pancreatic cystic neoplasms: a review of preoperative diagnosis and management first-author corresp ‡Corresponding Author ‡Corresponding Author †E-mail:liangtingbo@zju.edu.cn †E-mail:liangtingbo@zju.edu.cn liangtingbo@zju.edu.cn Department of Hepatobiliary-Pancreatic Surgery, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Department of Hepatobiliary-Pancreatic Surgery, the Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou 310009, China Pancreatic cystic neoplasms (PCNs) are a diverse group of neoplasms in the pancreas, and are more increasingly encountered with widespread abdominal screening and improved imaging techniques. The most common types of PCNs are serous cystic neoplasms (SCNs), mucinous cystic neoplasms (MCNs), and intraductal papillary mucinous neoplasms (IPMNs). Clinicians frequently feel bewildered in the differential diagnosis and subsequent management among the various types of lesions in the pancreas, which may lead to overtreatment or delayed treatment. The current review provides recent developments in the understanding of the three most common types of PCNs, the latest modalities used in preoperative diagnosis and differential diagnosis, as well as the most up to date management. Suggestions for diagnosis and differential diagnosis of SCNs, MCNs, and IPMNs are also provided for young surgeons. Better understanding of these neoplasms is essential for clinicians to make accurate diagnosis and to provide the best management for patients. Pancreatic cystic neoplasms Serous cystic neoplasms Intraductal papillary mucinous neoplasms Mucinous cystic neoplasms Diagnosis Introduction 1 With the widespread use of advanced radiological and endoscopic techniques, an increasing number of patients are being diagnosed with pancreatic cystic lesions. The incidence of pancreatic cystic lesions is about 1 in 100 hospitalized patients (Khalid and Brugge, 2007 2007 ). These lesions can be caused by injury, infection, or congenital anomalies. Pancreatic cystic neoplasms (PCNs) account for 10% to 15% of all pancreatic cystic lesions (Garcea et al., 2008 2008 ). PCNs are a diverse group of neoplasms, including serous cystic neoplasms (SCNs), mucinous cystic neoplasms (MCNs), intraductal papillary mucinous neoplasms (IPMNs), mucin solid pseudopapillary neoplasms, cystic endocrine neoplasms, and acinar cell cystadenocarcinomas. For clinicians, a reliable diagnosis and the subsequent management of PCNs are critical. When the cystic lesion is large or when the mass-related symptoms exist, surgical resection is recommended even when the diagnosis is uncertain. However, a majority of PCNs are asymptomatic and are found incidentally. These are challenging to preoperatively distinguish among the various types of lesions and to identify whether these lesions are benign, malignant, or potentially malignant. Therefore, an exact diagnosis is very important to avoid overtreatment or missing the opportunity to cure. Approximately 90% of PCNs are SCNs and mucin-producing neoplasms (MCNs and IPMNs) (Brugge et al., 2004a 2004a ). In this review we focus on clinical features, preoperative differential diagnostic strategies, and management of these three types of PCNs. We aim to provide updated knowledge about these three most common types of PCNs and to provide helpful recommendations on the appropriate treatment. Clinical features 1 Serous cystic neoplasms (SCNs) 2 SCNs mainly occur in women (70% to 75%), with the highest incidence in women in their 60s (Solcia et al., 1997 1997 ; Tseng et al., 2005 2005 ). SCNs can occur anywhere in the pancreas. A multicenter study of SCNs in Japan showed that the tumors were located in pancreatic head (39%), body (35%), tail (22%), and uncinate process (3%) (Kimura et al., 2012 2012 ). Although a majority of the patients with SCNs are asymptomatic, the most common presenting symptom is abdominal pain (25%), followed by palpable mass, jaundice, fatigue, and malaise (Tseng et al., 2005 2005 ). SCNs are broadly classified into microcystic adenomas and macrocystic (oligocystic) adenomas and are usually less than 2 cm (Buck and Hayes, 1990 1990 ). Serous microcystic adenomas (SMAs) are most common and they are mostly benign. These glycogen-rich cystadenomas are surrounded by fibrous capsules which separate them from the normal tissue (Buck and Hayes, 1990 1990 ). In the interior they form a honeycomb appearance, or a sponge-like structure with numerous small closely packed cysts arranged around a central stellate calcified scar (Buck and Hayes, 1990 1990 ). There are very few cases of microcystic adenocarcinomas reported (Abe et al., 1998 1998 ; Eriguchi et al., 1998 1998 ; Wu et al., 1999 1999 ). Histologically both microcystic adenomas and microcystic adenocarcinomas are identical and the presence of metastasis in liver or lymph nodes is the only way to differentiate them. Serous macrocystic adenomas are rare and have fewer and larger cysts. The cysts are often more than 1 cm (Kimura et al., 2012 2012 ). They mostly occur in the pancreatic head and, since they have a large size, they cause obstructive symptoms, including jaundice. von Hippel-Lindau disease, an autosomal dominant genetic disease that has a germline mutation in von Hippel-Lindau gene on chromosome 3p25 (Yoon and Brugge, 2012 2012 ), is also associated with serous macrocystic adenomas of the pancreas (Mohr et al., 2000 2000 ). The patients often have their entire pancreas involved and can have lesions on the central nervous system, kidneys, adrenal glands, and reproductive adnexal organs (Lonser et al., 2003 2003 ). Mucinous cystic neoplasms (MCNs) 2 MCNs are far more common in women (female: male, 20:1) and usually occur in the perimenopausal women (Goh et al., 2005 2005 ; 2006 2006 ). The incidence peaks in the 5th decade of their life (Yoon and Brugge, 2012 2012 ). In a study on 56 patients with MCNs, all were found to be women (Zamboni et al., 1999 1999 ). They are usually found in the tail or body of the pancreas, with only 5% to 10% in the head (Zamboni et al., 1999 1999 ). The clinical symptoms are non-specific, including abdominal pain and weight loss; however, most of the patients are asymptomatic and are found incidentally. Macroscopically MCNs are single spherical mass which may be unilocular or multilocular. They are frequently lined by mucinous epithelium and are supported by cellular, ovarian-like stroma. The identification of the feature is necessary to make the diagnosis of MCNs. Unlike IPMNs, MCNs do not have a connection with the pancreatic duct, unless there is fistula formation (Zamboni et al., 2010 2010 ). The World Health Organization (WHO) has classified MCNs into three different pathologies: adenomas, borderline tumors, and carcinomas (Zamboni et al., 2010 2010 ; Yoon and Brugge, 2012 2012 ). The columnar epithelial cells of the adenomatous tumors have abundant cytoplasm, basally located nuclei, have mild dysplasia, and are not mitotic. Borderline tumors show a moderate dysplasia, increased nuclei atypia, increased papillary projections and mitosis. MCNs of carcinoma type have multilayered epithelium forming papillary projections with high grade dysplasia, severe nuclear atypia, and increased mitosis. This type is further divided into non-invasive and invasive subtypes, with the presence or absence of malignant cells outside the cyst lining (Zamboni et al., 2010 2010 ). Most adenomatous type is unilocular and carcinomas are multilocular with papillary projections and mural nodules (Zamboni et al., 2010 2010 ). Intraductal papillary mucinous neoplasms (IPMNs) 2 IPMNs are neoplasms of mucinous producing epithelial cells and communicate with the pancreatic ducts (Fig. 1 1 ). They can arise in the main pancreatic duct and/or its branches (Adsay et al., 2010 2010 ). In Ohhashi et al. ( 1982 1982 )’s study, the first four cases of IPMNs were reported and they had a triad of mucus secretion, main pancreatic duct dilation, and a swollen duodenal papilla. IPMN was previously known as mucinous producing carcinoma, mucinous hypersecreting carcinoma, and intraductal mucinous producing tumor among others (Longnecker and Adler, 2000 2000 ). Currently the preferred name is intraductal papillary mucinous neoplasm (Xiao, 2012 2012 ), and is classified as IPMN with low or intermediate dysplasia, IPMN with high grade dysplasia, and IPMN with an associated invasive carcinoma (Adsay et al., 2010 2010 ). (a) Computed tomography (CT) scan shows a 2-cm size of cyst at the neck of pancreas with the dilation of main pancreatic duct. (b) During operation, cystic lesion was seen in the neck of pancreas. (c) Regional pancreatic resection was done, and from specimen, cystic lesion was shown to communicate with the main pancreatic duct. Intraductal papillary mucinous neoplasm (IPMN) was confirmed by pathology IPMNs are most frequently seen in patients in their late 60s and 70s and are more prevalent in men (Sohn et al., 2004 2004 ). They are usually found in the head of the pancreas as a solitary cystic mass, but in 20% to 30% of the cases they may be multifocal. In 5% to 10% cases they may involve the pancreas diffusely (Campbell and Azadeh, 2008 2008 ). Most of the patients are found incidentally and are asymptomatic. In symptomatic patients the typical symptoms include abdominal pain, weight loss, jaundice, new onset diabetes, and pale stools; non-typical symptoms can include acute necrotizing pancreatitis and sepsis due to acute cholangitis (Tibayan et al., 2000 2000 ). IPMNs may lead to chronic pancreatitis in the surrounding tissues, so serum amylase and lipase levels might also be elevated. IPMNs have also been associated with other syndromes such as Peutz-Jeghers syndrome and familial adenomatous polyposis (Sato et al., 2001 2001 ; Maire et al., 2002 2002 ). Some authors suggested that 30% of IPMN patients have a history of extra pancreatic neoplasms particularly in the stomach, colon, or rectum (Sugiyama and Atomi, 1999 1999 ; Adsay et al., 2000 2000 ; Adsay, 2002 2002 ). IPMNs are divided according to their location, including the main duct (MD) IPMN, branch duct (BD) IPMN, and combined IPMN which involves both MD and its branches. MD IPMNs are thought to be more aggressive (Fig. 2 2 ) and have a higher malignant potential, while BD IPMNs are less aggressive (Sohn et al., 2004 2004 ). The presence of a solid or gelatinous nodule suggests an invasive carcinoma which might not be seen grossly, so an extensive sampling is mandatory in all patients. (a) Computed tomography (CT) findings of main branch type IPMN in the body of pancreas. The margin of the mass was unclear and the splenic vein was involved. Note mucus can enlarge main duct dramatically. (b) Cholangioscopy can go through the proximal part of main duct smoothly during operation. (c) Pathology demonstrated a combined IPMN with an associated invasive carcinoma Histologically, intraductal proliferation of columnar mucin-producing epithelial cells can be seen and the epithelial cells may be of four different types, including intestinal, gastric, pancreatobiliary, and oncocytic types (Furukawa et al., 2005 2005 ). These types are associated with different survival rates. Patients with gastric type have the best prognosis whereas patients with pancreatobiliary type have the worst (Furukawa et al., 2011 2011 ). Preoperative diagnosis 1 Computed tomography (CT) and magnetic resonance imaging (MRI) 2 An appropriate differential diagnostic modality is critical for patients with PCNs to avoid unnecessary surgery. The two noninvasive imaging modalities which have been most frequently used to evaluate pancreatic cysts are CT and MRI. On CT scans and MRI, SCNs can have two kinds of appearances. SMAs have a distinct honey comb-like appearance (Fig. 3 3 ). The typical lesion is numerous (usually more than 6) microcysts (less than 2 cm), which are closely packed and thin-walled (Khurana et al., 2003 2003 ). In the Japanese study mentioned before, the honeycomb appearance was noted in 75.0% by CT, 89.2% by ultrasound (US), 100% by endoscopic ultrasound (EUS), and 86.2% by MRI in the microcystic type lesions (Kimura et al., 2012 2012 ). They are sometimes arranged around a central calcified scar presenting in around 20% of the cases (Yoon and Brugge, 2012 2012 ). This so-called sunburst appearance is pathognomonic for SMA. Macrocystic or oligocystic adenomas are more difficult to differentiate from MCNs on CT. In the CT scans, macrocystic adenomas are presented as unilocular cystic lesions with lobulated contour without wall enhancement (Cohen-Scali et al., 2003 2003 ). They lack a central scar, as is seen in SMAs, and were proven not to be sensitive to imaging (Kimura et al., 2012 2012 ). von Hippel-Lindau disease multiple pancreatic cysts are found on CT and MRI (Iwamuro et al., 2012 2012 ). According to one study done in Japan on von Hippel-Lindau disease, 82% of the cases had multiple cysts in the pancreas (Iwamuro et al., 2012 2012 ). Magnetic resonant cholangiopancreatography (MRCP) can show the ductal relationship with the lesions, but is not so important in the diagnosis of SCNs. (a) A distinct honeycomb-like appearance on computed tomography (CT) scan (see arrow). (b) The lesion has numerous microcysts and does not communicate with main duct on magnetic resonant cholangiopancreatography MCNs on CT scan appear as large cysts usually greater than 2 cm and are less than 6 in number as compared to SCNs (Megibow, 2008 2008 ; Yoon and Brugge, 2012 2012 ). Thin septae can be seen on CT, especially after administration of intravenous contrast (Fig. 4 4 ). Egg-shell like calcifications, presenting at the periphery of the lesions, are suggestive of malignancy (Yoon and Brugge, 2012 2012 ). (a) Computed tomography (CT) findings. Note the cystic lesion at the pancreatic head with the dilation of biliary tract caused by the oppression of the cystic lesion. The late enhancement of septa can be seen (see arrow). (b) Magnetic resonant cholangiopancreatography shows that the cystic lesion does not communicate with main pancreatic duct (see arrow). The dilation of biliary tract is clearly found. For patients with suspected malignant pancreatic cystic neoplasms, in order to identify the relationship with adjacent vessels, CT angiography is strongly recommended. CT angiography is very helpful to evaluate the resectability preoperatively In IPMNs, CT and MRI are used to see the exact anatomic location of the lesions and to check for metastasis. BD IPMN shows multiple small cysts (usually 1 to 2 cm) and MD IPMN shows a dilated main duct (Grogan et al., 2001 2001 ). MRCP is better than CT in the diagnosis of IPMNs as it can show the cystic lesion communicating with the ducts more clearly. Endoscopic retrograde cholangiopancreatography (ERCP) 2 ERCP can show whether the cyst is communicating with the ductal system or not. It is not so important in the diagnosis of SCNs or MCNs but is often used for the diagnosis of IPMNs as it can clearly show the anatomy of the pancreatic ducts. It is also useful to detect any mucin that is being extruded from the ampulla which is pathognomonic for IPMNs (Mohr et al., 2000 2000 ; Yoon and Brugge, 2012 2012 ). In MD IPMNs, ERCP shows dilated ducts and a filling defect caused by mucin or the cysts themselves, while in BD IPMNs the involved branches show dilation and communicate with the main duct (Lim et al., 2001 2001 ). It has been reported that ERCP showed the communication of the cystic lesion with the pancreatic duct in 80.8% of the cases while MRCP showed 55.7%, CT showed 53.8%, and MRI showed 42.3% (Kawamoto et al., 2005 2005 ). Another advantage of ERCP is that cytological sampling can be required, though it is an invasive procedure. Endoscopic ultrasound (EUS) and fine needle aspiration (FNA) 2 EUS is widely used to perform FNA in order to provide fluid for cytological analysis and tumor markers or amylase testing, which plays an important role in diagnosis of PCNs (Penman and Lennon, 2011 2011 ). It can also be used to analyze morphological features of PCNs. In EUS, multiple small anechoic cysts with thin septations can be seen in SMAs. SCNs normally do not express carcinoembryonic antigen (CEA), carbohydrate antigen (CA) 19-9, CA 125, etc. (Lewandrowski et al., 1993 1993 ). Amylase is also not easy to find because SCNs almost never communicate with the ductal system of the pancreas. In EUS, MCNs have thin-walled, fluid-filled large cysts, and very large cysts with irregular walls are highly suggestive of malignancy (Levy, 2009 2009 ). CEA in MCNs is high because of the production by mucinous epithelium. Other tumor markers like CA 15-3 and CA 72-4 are also high (Brugge et al., 2004b 2004b ). These features can be used to differentiate MCNs from SCNs, but not from IPMNs since they have the same markers elevated. Amylase is not present because MCNs do not communicate with the ducts. The main EUS finding which may suggest malignancy for MD IPMNs is dilatation of main duct greater than 10 mm, and for BD IPMNs malignancy is suggested by large tumors (greater than 4 cm) with irregular septa (Kubo et al., 2001 2001 ). Large mural nodules (greater than 10 mm) are also associated with malignancy in both types (Kubo et al., 2001 2001 ). CEA and other tumor markers are usually high but this makes it difficult to distinguish from MCNs alone. Amylase in the fluid is also high because of the lesions’ communication with the ducts. Contrast enhanced ultrasound (CEUS) 2 CEUS is a relatively new technique, currently used for diagnosis of hepatobiliary and pancreatic tumors. It has led to major improvements in the diagnostic capabilities of conventional US (D′Onofrio et al., 2010 2010 ). For PCNs, CEUS gives a good view of the septa and mural nodules. SCNs appear as a well delineated mass with small cysts inside. Following contrast administration, septa are enhanced, with the tumor showing a mulberry-like or honeycomb pattern. MCNs are characterized by cystic areas separated by septa, with parietal nodules and papillary wall protrusions. The parietal nodules might not be detected by conventional US due to the rich mucinous content. IPMNs are rarely detected by US. They appear as a non-homogeneous mass just below a dilated duct when they are large. Contrast agents might show the intraductal growth (Badea et al., 2009 2009 ). However, in most PCN cases, CEUS does not add significant diagnostic information; therefore CT scans and other diagnostic modalities are always necessary for their proper characterization (Recaldini et al., 2008 2008 ). Fluorodeoxyglucose positron emission tomography (FDG-PET)/computed tomography (CT) 2 FDG-PET is a sensitive and specific imaging protocol for the diagnosis and staging of several types of malignancies including pancreatic cancers (Nakamoto et al., 2000 2000 ; Pakzad et al., 2006 2006 ). It has been recognized that FDG-PET/CT is more sensitive than conventional imaging in the diagnosis of both primary pancreatic adenocarcinoma and associated distant metastases (Kauhanen et al., 2009 2009 ). FDG-PET/CT has also been used in distinguishing benign from malignant cystic lesions of the pancreas, and found to be more accurate than CT (Sperti et al., 2007 2007 ). It has been demonstrated that using FDG-PET/CT in combination with CT and tumor markers testing could improve the preoperative diagnostic sensitivity and accuracy in the patients with pancreatic cystic lesions. Thus FDG-PET/CT is instructive to manage PCNs. A positive result on FDG-PET strongly suggests malignancy and, therefore, a need for resection. A negative result shows a benign tumor that may be treated with limited resection or, in selected high-risk patients, with biopsy, follow-up, or both (Sperti et al., 2001 2001 ; 2005 2005 ; Mansour et al., 2006 2006 ). Management 1 Surgery 2 Until today, surgery is considered the best management option for PCNs. It has a number of benefits, including long term survival of patients, relief of symptoms, and diagnostic certainty (Roggin et al., 2010 2010 ). A study showed that out of 73 patients with PCN who underwent surgery, 70 patients survived and had a low rate of surgical complications (Sheehan et al., 2003 2003 ). Management of patients with PCNs depends on whether the lesions are mucinous or non-mucinous. Since SCNs are mostly benign, the prognosis is excellent. The incidence of serous cystadenocarcinoma is very low (Abe et al., 1998 1998 ; Eriguchi et al., 1998 1998 ; Wu et al., 1999 1999 ). The multicenter study of SCNs in Japan showed that out of 172 patients with SCNs only 2 had liver metastasis (Kimura et al., 2012 2012 ). Therefore the management is mostly to monitor and observe the patient. There are some exceptions to this rule and surgery should be considered for patients with symptoms (e.g., abdominal pain, mass effect, jaundice, and nausea), tumor size greater than 4 cm, or if the diagnosis is uncertain (Sakorafas et al., 2011 2011 ). Observation is also recommended for SCNs in elderly patients with typical signs on CT scan and smaller size of the lesion (Kimura et al., 2012 2012 ). The type of surgery depends on where the cystic lesion lies as SCNs can occur in any part of the pancreas. If it is in the proximal part of the pancreas Whipple’s procedure or pylorus-preserving pancreaticoduodenectomy is recommended (Warshaw et al., 1998 1998 ; Roggin et al., 2010 2010 ). A duodenum preserving resection of the pancreatic head is also suitable for lesions in the proximal part (Schwarz and Beger, 1996 1996 ). This procedure has a better outcome compared to Whipple’s procedure. If the tumor is in the distal part of the pancreas a distal pancreatectomy is performed (Warshaw et al., 1998 1998 ), and if it is not invasive, the spleen is often preserved. The tumor lying in the middle of the pancreas is removed by performing middle pancreatectomy (Warshaw et al., 1998 1998 ). Total pancreatectomy is seldom performed for SCNs as its mortality is very high. For mucinous neoplasms including IPMNs and MCNs, a more aggressive approach is required. MCNs are always surgically removed, because around 30% cases of MCNs are malignant (Reddy et al., 2004 2004 ) (Fig. 5 5 ). In a study done by Loyola University, 5 out of 20 MCN patients had mucinous cystadenocarcinoma (Sheehan et al., 2003 2003 ). Distal pancreatectomy was performed in four patients and one pancreatoduodenectomy was done, without any mortality (Sheehan et al., 2003 2003 ). In mucinous cystic adenoma, most patients had a distal pancreatectomy performed and the mortality was 0% in this study (Sheehan et al., 2003 2003 ). Whipple’s procedure was done. (a) Whipple’s specimen; (b) Pancreatic head cystic lesion being malignant (see arrow) IPMNs have an even greater chance of being malignant (around 60% for MD IPMNs and 30% for BD IPMNs) (Tanaka et al., 2006 2006 ). Most IPMNs are located in the head of the pancreas, and therefore a pancreatoduodenectomy is needed. IPMNs grow longitudinally in the ducts, so a negative margin needs to be established by a frozen section to confirm clearance of the tumor. If a clear negative margin cannot be established, a total pancreatectomy is performed (Brugge et al., 2004a 2004a ). In the study mentioned above, of the 18 patients who were surgically treated for IPMN, 11 patients had pancreatoduodenectomy, 5 patients had distal pancreatectomy and 2 patients had total pancreatectomy performed (Sheehan et al., 2003 2003 ). However, the mortality was higher for IPMN cases and 2 patients who underwent total pancreatectomy died (Sheehan et al., 2003 2003 ). In another study, the 5-year survival rate of IPMNs with invasive cancer was over 50% after resection (Chari et al., 2002 2002 ). For the mucinous neoplasms, Sendai Consensus Guidelines suggests surgical resection when there are symptoms associated with the cyst, the main pancreatic duct is dilated more than 10 mm, cyst size is more than 30 mm, intramural nodules are present, or cyst fluid cytology is positive or suggestive of malignancy (Tanaka et al., 2006 2006 ). The most common complication after surgery for PCN is pancreatic fistula. Other complications include abscess formation, wound infection, hemorrhage and other various complications like stroke, deep venous thrombosis, urinary tract infections, and delayed gastric emptying (Sheehan et al., 2003 2003 ). In the study mentioned before, 10% patients had fistula formation, all of which closed spontaneously (Sheehan et al., 2003 2003 ). Cyst ablation 2 Cyst ablation is a relatively new and less invasive method than surgery. This is particularly useful in patients who have high risks for surgery. It is an EUS-guided injection of ethanol or other ablative agents into the cavity of the cyst. Histological evidence of epithelial ablation in resected cysts has been showed and there was very few complications reported post procedure (Gan et al., 2005 2005 ). Recently, paclitaxel has been added to increase the ablative capacity of this procedure and was called EUS-guided ethanol lavage with paclitaxel injection (EUS-ELPI) (Yoon and Brugge, 2012 2012 ). In one study 52 patients underwent EUS-ELPI, 43 patients were followed up and a complete response was seen in 29 patients (Oh et al., 2011 2011 ). Cyst ablation is still in the experimental stage but seems a promising method in the future. Summary 1 Owing to the increased knowledge of clinical, endoscopic, and radiographic characteristics of PCNs, we can now better diagnose the most common types of PCNs preoperatively. This provides great information for further management of patients, including surgeries, follow-ups, or other procedures like cyst ablation. Since surgery itself increases a patient’s mortality and morbidity, a balance is required as when to perform surgery or observe the patient. Therefore careful diagnosis as to whether a lesion is benign, malignant or has a malignant potential is required, and clinicians need to assess whether a patient is at high risk or low risk for surgery. Keeping in mind all these factors, we should manage the patient accordingly. References H Abe , K Kubota , M Mori , K Miki , M Minagawa , T Noie , W Kimura , M Makuuchi . Serous cystadenoma of the pancreas with invasive growth: benign or malignant? . Am J Gastroenterol , 1998 . 93 ( 10 ): 1963 - 1966 . DOI: 10.1111/j.1572-0241.1998.00556.x http://doi.org/10.1111/j.1572-0241.1998.00556.x . Serous cystadenoma of the pancreas with invasive growth: benign or malignant? NV Adsay . Intraductal papillary mucinous neoplasms of the pancreas: pathology and molecular genetics . J Gastrointest Surg , 2002 . 6 ( 5 ): 656 - 659 . 12399051 DOI: 10.1016/S1091-255X(02)00057-4 http://doi.org/10.1016/S1091-255X(02)00057-4 . Intraductal papillary mucinous neoplasms of the pancreas: pathology and molecular genetics NV Adsay , DS Longnecker , DS Klimstra . Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms . Semin Diagn Pathol , 2000 . 17 ( 1 ): 16 - 30 . 10721804 . Pancreatic tumors with cystic dilatation of the ducts: intraductal papillary mucinous neoplasms and intraductal oncocytic papillary neoplasms NV Adsay , N Fukushima , T Furukawa , 等 . FT Bosman , F Carneiro , RH Hruban , 等 . Intraductal Neoplasms of the Pancreas . World Health Organization Classification of Tumours of the Digestive System , 2010 . 4th Ed : Lyon IARC , 304 - 313 . . Intraductal Neoplasms of the Pancreas R Badea , A Seicean , B Diaconu , R Stan-Iuga , Z Sparchez , M Tantau , M Socaciu . Contrast-enhanced ultrasound of the pancreas—a method beyond its potential or a new diagnostic standard? . J Gastrointestin Liver Dis , 2009 . 18 ( 2 ): 237 - 242 . . Contrast-enhanced ultrasound of the pancreas—a method beyond its potential or a new diagnostic standard? WR Brugge , GY Lauwers , D Sahani , C Fernandez-del Castillo , AL Warshaw . Cystic neoplasms of the pancreas . N Engl J Med , 2004 . 351 ( 12 ): 1218 - 1226 . 15371579 DOI: 10.1056/NEJMra031623 http://doi.org/10.1056/NEJMra031623 . Cystic neoplasms of the pancreas WR Brugge , K Lewandrowski , E Lee-Lewandrowski , BA Centeno , T Szydlo , S Regan , C Fernandez-del Castillo , AL Warshaw . Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study . Gastroenterology , 2004 . 126 ( 5 ): 1330 - 1336 . 15131794 DOI: 10.1053/j.gastro.2004.02.013 http://doi.org/10.1053/j.gastro.2004.02.013 . Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study JL Buck , WS Hayes . Microcystic adenoma of the pancreas . Radiographics , 1990 . 10 ( 2 ): 313 - 322 . 2183300 . Microcystic adenoma of the pancreas F Campbell , B Azadeh . Cystic neoplasms of the exocrine pancreas . Histopathology , 2008 . 52 ( 5 ): 539 - 551 . 17903202 DOI: 10.1111/j.1365-2559.2007.02856.x http://doi.org/10.1111/j.1365-2559.2007.02856.x . Cystic neoplasms of the exocrine pancreas ST Chari , D Yadav , TC Smyrk , EP DiMagno , LJ Miller , M Raimondo , JE Clain , IA Norton , RK Pearson , BT Petersen , 等 . Study of recurrence after surgical resection of intraductal papillary mucinous neoplasm of the pancreas . Gastroenterology , 2002 . 123 ( 5 ): 1500 - 1507 . 12404225 DOI: 10.1053/gast.2002.36552 http://doi.org/10.1053/gast.2002.36552 . Study of recurrence after surgical resection of intraductal papillary mucinous neoplasm of the pancreas F Cohen-Scali , V Vilgrain , G Brancatelli , P Hammel , MP Vullierme , A Sauvanet , Y Menu . Discrimination of unilocular macrocystic serous cystadenoma from pancreatic pseudocyst and mucinous cystadenoma with CT: initial observations . Radiology , 2003 . 228 ( 3 ): 727 - 733 . 12954892 DOI: 10.1148/radiol.2283020973 http://doi.org/10.1148/radiol.2283020973 . Discrimination of unilocular macrocystic serous cystadenoma from pancreatic pseudocyst and mucinous cystadenoma with CT: initial observations M D′Onofrio , A Gallotti , F Principe , RP Mucelli . Contrast-enhanced ultrasound of the pancreas . World J Radiol , 2010 . 2 ( 3 ): 97 - 102 . 21160942 DOI: 10.4329/wjr.v2.i3.97 http://doi.org/10.4329/wjr.v2.i3.97 . Contrast-enhanced ultrasound of the pancreas N Eriguchi , S Aoyagi , T Nakayama , M Hara , T Miyazaki , R Kutami , A Jimi . Serous cystadenocarcinoma of the pancreas with liver metastases . J Hepatobil Pancreat Surg , 1998 . 5 ( 4 ): 467 - 470 . DOI: 10.1007/s005340050075 http://doi.org/10.1007/s005340050075 . Serous cystadenocarcinoma of the pancreas with liver metastases T Furukawa , G Kloppel , N Volkan Adsay , J Albores-Saavedra , N Fukushima , A Horii , RH Hruban , Y Kato , DS Klimstra , DS Longnecker , 等 . Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study . Virchows Arch , 2005 . 447 ( 5 ): 794 - 799 . 16088402 DOI: 10.1007/s00428-005-0039-7 http://doi.org/10.1007/s00428-005-0039-7 . Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study T Furukawa , T Hatori , I Fujita , M Yamamoto , M Kobayashi , N Ohike , T Morohoshi , S Egawa , M Unno , S Takao , 等 . Prognostic relevance of morphological types of intraductal papillary mucinous neoplasms of the pancreas . Gut , 2011 . 60 ( 4 ): 509 - 516 . 21193453 DOI: 10.1136/gut.2010.210567 http://doi.org/10.1136/gut.2010.210567 . Prognostic relevance of morphological types of intraductal papillary mucinous neoplasms of the pancreas SI Gan , CC Thompson , GY Lauwers , BC Bounds , WR Brugge . Ethanol lavage of pancreatic cystic lesions: initial pilot study . Gastrointest Endosc , 2005 . 61 ( 6 ): 746 - 752 . 15855986 DOI: 10.1016/S0016-5107(05)00320-2 http://doi.org/10.1016/S0016-5107(05)00320-2 . Ethanol lavage of pancreatic cystic lesions: initial pilot study G Garcea , SL Ong , A Rajesh , CP Neal , CA Pollard , DP Berry , AR Dennison . Cystic lesions of the pancreas: a diagnostic and management dilemma . Pancreatology , 2008 . 8 ( 3 ): 236 - 251 . 18497542 DOI: 10.1159/000134279 http://doi.org/10.1159/000134279 . Cystic lesions of the pancreas: a diagnostic and management dilemma BK Goh , YM Tan , MP Kumarasinghe , LL Ooi . Mucinous cystic tumor of the pancreas with ovarian-like mesenchymal stroma in a male patient . Dig Dis Sci , 2005 . 50 ( 11 ): 2170 - 2177 . 16240235 DOI: 10.1007/s10620-005-3027-5 http://doi.org/10.1007/s10620-005-3027-5 . Mucinous cystic tumor of the pancreas with ovarian-like mesenchymal stroma in a male patient BK Goh , YM Tan , YF Chung , PK Chow , PC Cheow , WK Wong , LL Ooi . A review of mucinous cystic neoplasms of the pancreas defined by ovarian-type stroma: clinicopathological features of 344 patients . World J Surg , 2006 . 30 ( 12 ): 2236 - 2245 . 17103100 DOI: 10.1007/s00268-006-0126-1 http://doi.org/10.1007/s00268-006-0126-1 . A review of mucinous cystic neoplasms of the pancreas defined by ovarian-type stroma: clinicopathological features of 344 patients JR Grogan , K Saeian , AJ Taylor , F Quiroz , MJ Demeure , RA Komorowski . Making sense of mucin-producing pancreatic tumors . Am J Roentgenol , 2001 . 176 ( 4 ): 921 - 929 . . Making sense of mucin-producing pancreatic tumors M Iwamuro , H Kawamoto , H Shiraha , H Nose , K Yamamot . Pancreatic involvement in 11 cases of von Hippel-Lindau disease . Hepatogastroenterology , 2012 . 59 ( 114 ): 589 - 591 . 22353527 . Pancreatic involvement in 11 cases of von Hippel-Lindau disease SP Kauhanen , G Komar , MP Seppänen , KI Dean , HR Minn , SA Kajander , I Rinta-Kiikka , K Alanen , RJ Borra , PA Puolakkainen , 等 . A prospective diagnostic accuracy study of 18F-fluorodeoxyglucose positron emission tomography/computed tomography, multidetector row computed tomography, and magnetic resonance imaging in primary diagnosis and staging of pancreatic cancer . Ann Surg , 2009 . 250 ( 6 ): 957 - 963 . 19687736 DOI: 10.1097/SLA.0b013e3181b2fafa http://doi.org/10.1097/SLA.0b013e3181b2fafa . A prospective diagnostic accuracy study of 18F-fluorodeoxyglucose positron emission tomography/computed tomography, multidetector row computed tomography, and magnetic resonance imaging in primary diagnosis and staging of pancreatic cancer S Kawamoto , KM Horton , LP Lawler , RH Hruban , EK Fishman . Intraductal papillary mucinous neoplasm of the pancreas: can benign lesions be differentiated from malignant lesions with multidetector CT? . Radiographics , 2005 . 25 ( 6 ): 1451 - 1470 . 16284127 DOI: 10.1148/rg.256055036 http://doi.org/10.1148/rg.256055036 . Intraductal papillary mucinous neoplasm of the pancreas: can benign lesions be differentiated from malignant lesions with multidetector CT? A Khalid , W Brugge . ACG practice guidelines for the diagnosis and management of neoplastic pancreatic cysts . Am J Gastroenterol , 2007 . 102 ( 10 ): 2339 - 2349 . 17764489 DOI: 10.1111/j.1572-0241.2007.01516.x http://doi.org/10.1111/j.1572-0241.2007.01516.x . ACG practice guidelines for the diagnosis and management of neoplastic pancreatic cysts B Khurana , KJ Mortele , J Glickman , SG Silverman , PR Ros . Macrocystic serous adenoma of the pancreas: radiologic-pathologic correlation . Am J Roentgenol , 2003 . 181 ( 1 ): 119 - 123 . . Macrocystic serous adenoma of the pancreas: radiologic-pathologic correlation W Kimura , T Moriya , K Hanada , H Abe , A Yanagisawa , N Fukushima , N Ohike , M Shimizu , T Hatori , N Fujita , 等 . Multicenter study of SCN of the Japan pancreas society: a multi-institutional study of 172 patients . Pancreas , 2012 . 41 ( 3 ): 380 - 387 . 22415666 DOI: 10.1097/MPA.0b013e31822a27db http://doi.org/10.1097/MPA.0b013e31822a27db . Multicenter study of SCN of the Japan pancreas society: a multi-institutional study of 172 patients H Kubo , Y Chijiiwa , K Akahoshi , S Hamada , N Harada , T Sumii , M Takashima , H Nawata . Intraductal papillary-mucinous tumors of the pancreas: differential diagnosis between benign and malignant tumors by endoscopic ultrasonography . Am J Gastroenterol , 2001 . 96 ( 5 ): 1429 - 1434 . 11374678 DOI: 10.1111/j.1572-0241.2001.03794.x http://doi.org/10.1111/j.1572-0241.2001.03794.x . Intraductal papillary-mucinous tumors of the pancreas: differential diagnosis between benign and malignant tumors by endoscopic ultrasonography MJ Levy . Pancreatic cysts . Gastrointest Endosc , 2009 . 69 ( Suppl. 2 ): 110 - 116 . DOI: 10.1016/j.gie.2008.12.011 http://doi.org/10.1016/j.gie.2008.12.011 . Pancreatic cysts KB Lewandrowski , JF Southern , MR Pins , CC Compton , AL Warshaw . Cyst fluid analysis in the differential diagnosis of pancreatic cysts: a comparison of pseudocysts, serous cystadenomas, mucinous cystic neoplasms, and mucinous cystadenocarcinoma . Ann Surg , 1993 . 217 ( 1 ): 41 - 47 . 8424699 DOI: 10.1097/00000658-199301000-00008 http://doi.org/10.1097/00000658-199301000-00008 . Cyst fluid analysis in the differential diagnosis of pancreatic cysts: a comparison of pseudocysts, serous cystadenomas, mucinous cystic neoplasms, and mucinous cystadenocarcinoma JH Lim , G Lee , YL Oh . Radiologic spectrum of intraductal papillary mucinous tumor of the pancreas . Radiographics , 2001 . 21 ( 2 ): 323 - 337 . 11259696 . Radiologic spectrum of intraductal papillary mucinous tumor of the pancreas DS Longnecker , G Adler . SR Hamilton , LA Aaltonen . Intraductal Papillary-Mucinous Neoplasms . Pathology and Genetics of Tumours of the Digestive System. World Health Organization Classification of Tumours , 2000 . : Lyon IARC , 237 - 240 . . Intraductal Papillary-Mucinous Neoplasms RR Lonser , GM Glenn , M Walther , EY Chew , SK Libutti , WM Linehan , EH Oldfield . von Hippel-Lindau disease . Lancet , 2003 . 361 ( 9374 ): 2059 - 2067 . 12814730 DOI: 10.1016/S0140-6736(03)13643-4 http://doi.org/10.1016/S0140-6736(03)13643-4 . von Hippel-Lindau disease F Maire , P Hammel , B Terris , S Olschwang , D O′Toole , A Sauvanet , L Palazzo , P Ponsot , B Laplane , P Levy , 等 . Intraductal papillary and mucinous pancreatic tumour: a new extracolonic tumour in familial adenomatous polyposis . Gut , 2002 . 51 ( 3 ): 446 - 449 . 12171972 DOI: 10.1136/gut.51.3.446 http://doi.org/10.1136/gut.51.3.446 . Intraductal papillary and mucinous pancreatic tumour: a new extracolonic tumour in familial adenomatous polyposis JC Mansour , L Schwartz , N Pandit-Taskar , M D′Angelica , Y Fong , SM Larson , MF Brennan , PJ Allen . The utility of F-18 fluorodeoxyglucose whole body PET imaging for determining malignancy in cystic lesions of the pancreas . J Gastrointest Surg , 2006 . 10 ( 10 ): 1354 - 1360 . 17175454 DOI: 10.1016/j.gassur.2006.08.002 http://doi.org/10.1016/j.gassur.2006.08.002 . The utility of F-18 fluorodeoxyglucose whole body PET imaging for determining malignancy in cystic lesions of the pancreas AJ Megibow . RM Gore , MS Levine . Pancreatic Neoplams . Textbook of Gastrointestinal Radiology , 2008 . 3rd Ed : Philadelphia Elsevier Saunders , 1915 - 1931 . DOI: 10.1016/B978-1-4160-2332-6.50105-6 http://doi.org/10.1016/B978-1-4160-2332-6.50105-6 . Pancreatic Neoplams VH Mohr , AO Vortmeyer , Z Zhuang , SK Libutti , MM Walther , PL Choyke , B Zbar , WM Linehan , IA Lubensky . Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients . Am J Pathol , 2000 . 157 ( 5 ): 1615 - 1621 . 11073821 DOI: 10.1016/S0002-9440(10)64799-2 http://doi.org/10.1016/S0002-9440(10)64799-2 . Histopathology and molecular genetics of multiple cysts and microcystic (serous) adenomas of the pancreas in von Hippel-Lindau patients Y Nakamoto , T Higashi , H Sakahara , N Tamaki , M Kogire , R Doi , R Hosotani , M Imamura , J Konishi . Delayed (18)F-fluoro-2-deoxy- D -glucose positron emission tomography scan for differentiation between malignant and benign lesions in the pancreas . Cancer , 2000 . 89 ( 12 ): 2547 - 2554 . 11135214 DOI: 10.1002/1097-0142(20001215)89:12<2547::AID-CNCR5>3.0.CO;2-V http://doi.org/10.1002/1097-0142(20001215)89:12<2547::AID-CNCR5>3.0.CO;2-V . Delayed (18)F-fluoro-2-deoxy-D-glucose positron emission tomography scan for differentiation between malignant and benign lesions in the pancreas HC Oh , DW Seo , TJ Song , SH Moon , DH Park , SS Lee , SK Lee , MH Kim , J Kim . Endoscopic ultrasonography-guided ethanol lavage with paclitaxel injection treats patients with pancreatic cysts . Gastroenterology , 2011 . 140 ( 1 ): 172 - 179 . 20950614 DOI: 10.1053/j.gastro.2010.10.001 http://doi.org/10.1053/j.gastro.2010.10.001 . Endoscopic ultrasonography-guided ethanol lavage with paclitaxel injection treats patients with pancreatic cysts K Ohhashi , Y Murakami , M Maruyama . Four cases of mucin-producing cancer of the pancreas on specific findings of the papilla of Vater . Prog Dig Endosc , 1982 . 20 348 - 351 . . Four cases of mucin-producing cancer of the pancreas on specific findings of the papilla of Vater F Pakzad , AM Groves , PJ Ell . The role of positron emission tomography in the management of pancreatic cancer . Semin Nucl Med , 2006 . 36 ( 3 ): 248 - 256 . 16762614 DOI: 10.1053/j.semnuclmed.2006.03.005 http://doi.org/10.1053/j.semnuclmed.2006.03.005 . The role of positron emission tomography in the management of pancreatic cancer ID Penman , AM Lennon . RH Hawes , P Fockens , S Varadarajulu . EUS in the Evaluation of Pancreatic Cysts . Endosonography , 2011 . 2nd Ed : Philadelphia Elsevier Saunders , 166 - 177 . DOI: 10.1016/B978-1-4377-0805-9.00015-7 http://doi.org/10.1016/B978-1-4377-0805-9.00015-7 . EUS in the Evaluation of Pancreatic Cysts C Recaldini , G Carrafiello , E Bertolotti , MG Angeretti , C Fugazzola . Contrast-enhanced ultrasonograpic findings in pancreatic tumors . Int J Med Sci , 2008 . 5 ( 4 ): 203 - 208 . 18645620 DOI: 10.7150/ijms.5.203 http://doi.org/10.7150/ijms.5.203 . Contrast-enhanced ultrasonograpic findings in pancreatic tumors RP Reddy , TC Smyrk , M Zapiach , MJ Levy , RK Pearson , JE Clain , MB Farnell , MG Sarr , ST Chari . Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer . Clin Gastroenterol Hepatol , 2004 . 2 ( 11 ): 1026 - 1031 . 15551256 DOI: 10.1016/S1542-3565(04)00450-1 http://doi.org/10.1016/S1542-3565(04)00450-1 . Pancreatic mucinous cystic neoplasm defined by ovarian stroma: demographics, clinical features, and prevalence of cancer KK Roggin , J Chennat , A Oto , A Noffsinger , A Briggs , JB Matthews . Pancreatic cystic neoplasm . Curr Probl Surg , 2010 . 47 ( 6 ): 459 - 510 . 20451023 DOI: 10.1067/j.cpsurg.2010.02.002 http://doi.org/10.1067/j.cpsurg.2010.02.002 . Pancreatic cystic neoplasm GH Sakorafas , V Smyrniotis , KM Reid-Lombardo , MG Sarr . Primary pancreatic cystic neoplasms revisited. Part I: serous cystic neoplasms . Surg Oncol , 2011 . 20 ( 2 ): e84 - e92 . 21237638 DOI: 10.1016/j.suronc.2010.12.002 http://doi.org/10.1016/j.suronc.2010.12.002 . Primary pancreatic cystic neoplasms revisited. Part I: serous cystic neoplasms N Sato , C Rosty , M Jansen , N Fukushima , T Ueki , CJ Yeo , JL Cameron , CA Iacobuzio-Donahue , RH Hruban , M Goggins . STK11/LKB1 Peutz-Jeghers gene inactivation in intraductal papillary-mucinous neoplasms of the pancreas . Am J Pathol , 2001 . 159 ( 6 ): 2017 - 2022 . 11733352 DOI: 10.1016/S0002-9440(10)63053-2 http://doi.org/10.1016/S0002-9440(10)63053-2 . STK11/LKB1 Peutz-Jeghers gene inactivation in intraductal papillary-mucinous neoplasms of the pancreas A Schwarz , HG Beger . Modification of Duodenum-Preserving Pancreatic Head Resection with Segmental Resection of the Duodenum: Experience with Four Cases (Oral Presentation) . 1996 . Annual Meeting of the Vereinigung Mittelrheinischer . Chirurgen, Ulm . Modification of Duodenum-Preserving Pancreatic Head Resection with Segmental Resection of the Duodenum: Experience with Four Cases (Oral Presentation) MK Sheehan , K Beck , J Pickleman , GV Aranha . Spectrum of cystic neoplasms of the pancreas and their surgical management . Arch Surg , 2003 . 138 ( 6 ): 657 - 660 . 12799338 DOI: 10.1001/archsurg.138.6.657 http://doi.org/10.1001/archsurg.138.6.657 . Spectrum of cystic neoplasms of the pancreas and their surgical management TA Sohn , CJ Yeo , JL Cameron , RH Hruban , N Fukushima , KA Campbell , KD Lillemoe . Intraductal papillary mucinous neoplasms of the pancreas: an updated experience . Ann Surg , 2004 . 239 ( 6 ): 788 - 797 . 15166958 DOI: 10.1097/01.sla.0000128306.90650.aa http://doi.org/10.1097/01.sla.0000128306.90650.aa . Intraductal papillary mucinous neoplasms of the pancreas: an updated experience E Solcia , C Capella , G Kloppel . Tumors of the Pancreas. Atlas of Tumor Pathology , 1997 . : Washington DC AFIP , 103 - 114 . 3rd Series, Fascicle 20 . Tumors of the Pancreas. Atlas of Tumor Pathology C Sperti , C Pasquali , F Chierichetti , G Liessi , G Ferlin , S Pedrazzoli . Value of 18-fluorodeoxyglucose positron emission tomography in the management of patients with cystic tumors of the pancreas . Ann Surg , 2001 . 234 ( 5 ): 675 - 680 . 11685032 DOI: 10.1097/00000658-200111000-00014 http://doi.org/10.1097/00000658-200111000-00014 . Value of 18-fluorodeoxyglucose positron emission tomography in the management of patients with cystic tumors of the pancreas C Sperti , C Pasquali , G Decet , F Chierichetti , G Liessi , S Pedrazzoli . F-18-fluorodeoxyglucose positron emission tomography in differentiating malignant from benign pancreatic cysts: a prospective study . J Gastrointest Surg , 2005 . 9 ( 1 ): 22 - 28 . 15623441 DOI: 10.1016/j.gassur.2004.10.002 http://doi.org/10.1016/j.gassur.2004.10.002 . F-18-fluorodeoxyglucose positron emission tomography in differentiating malignant from benign pancreatic cysts: a prospective study C Sperti , S Bissoli , C Pasquali , L Frison , G Liessi , F Chierichetti , S Pedrazzoli . 18-Fluorodeoxyglucose positron emission tomography enhances computed tomography diagnosis of malignant intraductal papillary mucinous neoplasms of the pancreas . Ann Surg , 2007 . 246 ( 6 ): 932 - 937 . 18043094 DOI: 10.1097/SLA.0b013e31815c2a29 http://doi.org/10.1097/SLA.0b013e31815c2a29 . 18-Fluorodeoxyglucose positron emission tomography enhances computed tomography diagnosis of malignant intraductal papillary mucinous neoplasms of the pancreas M Sugiyama , Y Atomi . Extrapancreatic neoplasms occur with unusual frequency in patients with intraductal papillary mucinous tumors of the pancreas . Am J Gastroenterol , 1999 . 94 ( 2 ): 470 - 473 . 10022648 DOI: 10.1111/j.1572-0241.1999.879_h.x http://doi.org/10.1111/j.1572-0241.1999.879_h.x . Extrapancreatic neoplasms occur with unusual frequency in patients with intraductal papillary mucinous tumors of the pancreas M Tanaka , S Chari , V Adsay , C Fernandez-del Castillo , M Falconi , M Shimizu , K Yamaguchi , K Yamao , S Matsuno . International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas . Pancreatology , 2006 . 6 ( 1-2 ): 17 - 32 . 16327281 DOI: 10.1159/000090023 http://doi.org/10.1159/000090023 . International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas F Tibayan , M Vierra , B Mindelzun , D Tsang , J McClenathan , H Young , HW Trueblood . Clinical presentation of mucin-secreting tumors of the pancreas . Am J Surg , 2000 . 179 ( 5 ): 349 - 351 . 10930477 DOI: 10.1016/S0002-9610(00)00376-7 http://doi.org/10.1016/S0002-9610(00)00376-7 . Clinical presentation of mucin-secreting tumors of the pancreas JF Tseng , AL Warshaw , DV Sahani , GY Lauwers , DW Rattner , C Fernandez-del Castillo . Serous cystadenoma of the pancreas: tumor growth rates and recommendations for treatment . Ann Surg , 2005 . 242 ( 3 ): 413 - 419 . 16135927 . Serous cystadenoma of the pancreas: tumor growth rates and recommendations for treatment AL Warshaw , DW Rattner , C Fernandezdel-del Castillo , K Z′graggen . Middle segment pancreatectomy: a novel technique for conserving pancreatic tissue . Arch Surg , 1998 . 133 ( 3 ): 327 - 331 . 9517749 DOI: 10.1001/archsurg.133.3.327 http://doi.org/10.1001/archsurg.133.3.327 . Middle segment pancreatectomy: a novel technique for conserving pancreatic tissue CM Wu , EK Fishman , RK Hruban , WD Schlott , JL Cameron . Serous cystic neoplasm involving the pancreas and liver: an unusual clinical entity . Abdom Imaging , 1999 . 24 ( 1 ): 75 - 77 . 9933679 DOI: 10.1007/s002619900445 http://doi.org/10.1007/s002619900445 . Serous cystic neoplasm involving the pancreas and liver: an unusual clinical entity SY Xiao . Intraductal papillary mucinous neoplasm of the pancreas: an update . Scientifica , 2012 . 2012 893632 DOI: 10.6064/2012/893632 http://doi.org/10.6064/2012/893632 . Intraductal papillary mucinous neoplasm of the pancreas: an update WJ Yoon , WR Brugge . Pancreatic cystic neoplasms: diagnosis and management . Gastroenterol Clin North Am , 2012 . 41 ( 1 ): 103 - 118 . 22341252 DOI: 10.1016/j.gtc.2011.12.016 http://doi.org/10.1016/j.gtc.2011.12.016 . Pancreatic cystic neoplasms: diagnosis and management G Zamboni , A Scarpa , G Bogina , C Iacono , C Bassi , G Talamini , F Sessa , C Capella , E Solcia , F Rickaert , 等 . Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors . Am J Surg Pathol , 1999 . 23 ( 4 ): 410 - 422 . 10199470 DOI: 10.1097/00000478-199904000-00005 http://doi.org/10.1097/00000478-199904000-00005 . Mucinous cystic tumors of the pancreas: clinicopathological features, prognosis, and relationship to other mucinous cystic tumors G Zamboni , N Fukushima , RH Hruban . FT Bosman , F Carneiro , RH Hruban , 等 . Mucinous Cystic Neoplasms of the Pancreas . World Health Organization Classification of Tumours of the Digestive System , 2010 . 4th Ed : Lyon IARC , 300 - 303 . . Mucinous Cystic Neoplasms of the Pancreas 10.1631/jzus.B1200283 Project supported by the National Natural Science Foundation of China (Nos. 30925033, 30801101, and 81171884), and the Innovation and High-Level Talent Training Program of Department of Health of Zhejiang Province, China 2012-10-17 2013-01-30 2013-01-16 2022-01-05 Copyright © Zhejiang University and Springer-Verlag Berlin Heidelberg 2013 2013 Journal of Zhejiang University-SCIENCE B (Biomedicine and Biotechnology) 03 14