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Serous cystic neoplasms (SCNs) are benign cystic neoplasms that predominantly occur in the tail of the pancreas in elderly women. It is well known that patients with von Hippel-Lindau syndrome can develop SCNs and neuroendocrine tumors in the pancreas. However, our understanding on SCNs accompanying other pancreatic tumors (SCNAOPTs) is limited. We compared the clinicopathological features of 15 surgically resected SCNAOPTs with 259 conventional SCNs. The prevalence of SCNAOPT was 5%. The SCNAOPTs were significantly smaller than conventional solitary SCNs, and they were more commonly observed in the head of the pancreas, whereas conventional solitary SCNs were more frequently noted in the body and tail. However, no differences were found in terms of sex, patient age, or the gross patterns of the SCNs. Accompanying neoplasms included 7 intraductal papillary mucinous neoplasms, 1 colloid carcinoma arising from intraductal papillary mucinous neoplasm, 6 neuroendocrine tumors, and 1 solid pseudopapillary neoplasm. Four neuroendocrine tumors associated with von Hippel-Lindau syndrome occurred as multiples, whereas 2 neuroendocrine tumors without von Hippel-Lindau syndrome were solitary. In summary, SCNAOPTs comprise 5% of all SCNs and tend to be smaller and located in the head of the pancreas. Common accompanying tumors include intraductal papillary mucinous neoplasms, neuroendocrine tumors, and other neoplasms such as colloid carcinoma and solid pseudopapillary neoplasm., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Aims: Pancreatic cystic neoplasms (PCN) with malignant potential are thought to be less aggressive than ordinary ductal adenocarcinoma, even in the setting of malignant transformation. Therefore, deciding whether or not to carry out surgery is very difficult, especially in elderly and asymptomatic patients, because of the high risk of perioperative morbidities. The aim of the present study was to examine clinical outcomes of PCN patients aged 65 years or older., Methods: This retrospective analysis included patients with incidentally detected PCN with follow-up durations >1 year. Patients diagnosed with obvious simple cysts, pseudocysts or pancreatic cancer and patients with a history of pancreatic disease were excluded from the study., Results: The present study included 201 patients (older group 104 patients ≥65 years; younger group 97 patients <65 years). Surgical resections were carried out for 27 patients in the older group and 41 patients in the younger group. There were 133 patients who were followed up without surgery (mean follow-up duration 41 months). Postoperative morbidity occurred in 22.2% of the patients in the older group and 21.9% of the patients in the younger group. Malignancy occurred in one patient in the older group and in two patients in the younger group. The PCN diameter increased in 20 patients during follow up: 16.9% of the older group and 12.5% of the younger group., Conclusions: The malignancy rate was very low in incidental PCN patients irrespective of age. Follow-up observation without surgery appears to be a safe option in older patients with morbidity. Geriatr Gerontol Int 2017; 17: 256-261., (© 2016 Japan Geriatrics Society.)

Background & Aims: Santorinicele, a rare focal cystic dilation of the distal portion of the dorsal pancreatic duct at the minor papilla, can be a cause of recurrent acute pancreatitis (RAP). Endoscopic minor papilla sphincterotomy (EMPS) has been evaluated as a treatment in case reports but never systematically investigated., Methods: We performed a retrospective analysis of the efficacy of EMPS in reducing episodes of pancreatitis. We collected data on 30 patients with santorinicele and RAP who underwent EMPS from June 2009 through April 2015 at University Hospital of Verona in Italy. The mean follow-up period was 43.8 months., Results: The average number of pancreatitis episodes per year before EMPS was 1.59 vs 0.18 episodes after EMPS; the average number of pancreatitis cases that occurred during a comparable time period before EMPS was 2.63 vs 0.67 cases after EMPS (P < .0001). Complete responses to EMPS (no recurrence of pancreatitis) were reported for 80% of patients. Six patients relapsed after a mean time of 16 months. Five patients were found to have a potential cause of RAP beyond santorinicele (2 patients had post-sphincterotomy stenosis, 1 patient was a chronic consumer of alcohol, 1 patient had a mutation in the CFTR gene, and 1 patient had a side-branch intraductal papillary mucinous neoplasm)., Conclusions: EMPS is effective in reducing the incidence of pancreatitis in patients with santorinicele., (Copyright © 2017 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Hepatobiliopancreatic surgery is challenging becouse of the complexity of resections and reconstructions and variability of vascular anatomy. The arterial vascularization of the liver is susteined by the common hepatic artery (CHA) that originates from the celiac trunk (CT). The CHA bifurcates into the gastroduodenal artery (GDA) and proper hepatic artery (PHA) 0.5-1 cm medial to the common bile duct (CBD), and the PHA bifurcates into a right and a left branch at hepatic hilum. The most frequent variants are the right hepatic artery (RHA) from the superior mesenteric artery (SMA), the left hepatic artery (LHA) from the left gastric artery (LGA) or a combination of these two variants. Herein, we describe the case of a 70 years old woman affected by IPMN of the pancreas with a quadrifurcation of CHA, associated with a RHA off the SMA., Key Words: Anatomic variant Hepatic artery variant, Hepatic anatomy, Hepatic vascular anatomy.

Objective: Our objective was to develop an approach for selecting combinatorial markers of pathology from diverse clinical data types. We demonstrate this approach on the problem of pancreatic cyst classification., Materials and Methods: We analyzed 1026 patients with surgically resected pancreatic cysts, comprising 584 intraductal papillary mucinous neoplasms, 332 serous cystadenomas, 78 mucinous cystic neoplasms, and 42 solid-pseudopapillary neoplasms. To derive optimal markers for cyst classification from the preoperative clinical and radiological data, we developed a statistical approach for combining any number of categorical, dichotomous, or continuous-valued clinical parameters into individual predictors of pathology. The approach is unbiased and statistically rigorous. Millions of feature combinations were tested using 10-fold cross-validation, and the most informative features were validated in an independent cohort of 130 patients with surgically resected pancreatic cysts., Results: We identified combinatorial clinical markers that classified serous cystadenomas with 95% sensitivity and 83% specificity; solid-pseudopapillary neoplasms with 89% sensitivity and 86% specificity; mucinous cystic neoplasms with 91% sensitivity and 83% specificity; and intraductal papillary mucinous neoplasms with 94% sensitivity and 90% specificity. No individual features were as accurate as the combination markers. We further validated these combinatorial markers on an independent cohort of 130 pancreatic cysts, and achieved high and well-balanced accuracies. Overall sensitivity and specificity for identifying patients requiring surgical resection was 84% and 81%, respectively., Conclusions: Our approach identified combinatorial markers for pancreatic cyst classification that had improved performance relative to the individual features they comprise. In principle, this approach can be applied to any clinical dataset comprising dichotomous, categorical, and continuous-valued parameters., (© The Author 2016. Published by Oxford University Press on behalf of the American Medical Informatics Association. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Objectives: Pancreatic serous cystadenomas (SCAs) are benign tumors. Technological advances in imaging have led to increased recognition of asymptomatic pancreatic cysts, consequently increasing the demand for cytomorphologic evaluations of cyst fluid., Study Design: A retrospective search through the pathology archives over an 11-year period was performed to identify SCAs from pancreatectomy specimens with a presurgical pancreatic EUS-guided fine-needle aspiration (FNA)., Results: Fifty-one FNAs were identified. The average patient age was 59.9 years and 34 (67%) were female. Thirty-five (69%) of the SCAs were located in the body or tail of the pancreas. SCAs ranged in size from 1.3 to 8.0 cm (mean 4.9). On imaging, features suggestive of SCA were seen in 7 (14%) cases. The cytologic diagnoses were as follows: SCA in 5 (10%) cases, suspicious for mucin-producing neoplastic cyst in 4 (8%), pseudocyst in 4 (8%), and benign ductal and/or acinar epithelium, not otherwise specified in 24 (47%). Additionally, 14 (27%) cases were deemed nondiagnostic., Conclusions: A cytopathologic diagnosis of SCA on FNA is extremely difficult. The salient cytomorphologic features for identifying SCAs included scant cellularity, a mostly clear background, absence of extracellular mucin, hemosiderin-laden macrophages, and loose fragments of cuboidal cells with a notable absence of necrosis, atypia, and mitoses., (© 2016 S. Karger AG, Basel.)

Background: Pancreatic cystic lesions found in the literature are estimated between 2.6-19.6%. These neoplasms are almost exclusively benign tumors, and rarely become malignant. The aim of this paper was to report a feasible total pancreatectomy with portal resection in a giant pancreatic serous cystadenoma mimicking a malignant tumor., Case Presentation: A healthy 52-year-old female patient presented to us with a giant pancreatic cyst with unknown preoperative diagnosis and a secondary dilatation of the Wirsung duct, and unclear vascular invasion. The imaging assessment was not conclusive for malignancy. Furthermore, it was decided to perform a total pancreatectomy with portal vein resection and splenectomy. The pathological findings revealed a microcystic serous cystadenoma of 10 cm with abundant myxoid stromal degeneration and focal papillary proliferation without atypia., Conclusion: Unless the diagnosis of serous cystadenoma is certain, cystic tumors with inconclusive clinical and imaging features should be radically treated.

Background: In 2015, the American Gastroenterological Association (AGA) published guidelines to provide recommendations for management of suspected pancreatic cystic neoplasms (PCNs). The aim of this study was to compare efficacy of these with the Fukuoka consensus guidelines in predicting advanced neoplasia (AN) in patients with suspected PCNs., Study Design: We performed a retrospective study of 239 patients who underwent surgical resection for suspected mucinous PCN at a tertiary care center from 2000 to 2014. Surgical pathology was the gold standard. The AGA and Fukuoka criteria were applied, and their performance in predicting AN, defined as invasive cancer or high-grade dysplasia (HGD), was assessed., Results: Advanced neoplasia was found in 71 of 239 (29.7%) patients (28 invasive cancer, 43 HGD). The Fukuoka "high-risk" (FG-HR) and AGA "high-risk" (AGA-HR) criteria identified patients with AN with sensitivities of 28.2% and 35.2%, specificities of 95.8% and 94.0%, positive predictive values of 74.1% and 71.4%, and negative predictive values of 75.9% and 77.5%, respectively. Overall, there was no significant difference between the guidelines for prediction of AN. There were 7 and 6 cases with invasive cancer, and 23 and 24 cases with HGD missed by the FG-HR and AGA-HR guidelines, respectively., Conclusions: In a retrospective analysis, the AGA guidelines are not superior to the Fukuoka guidelines in identifying AN in suspected PCNs. Both sets of guidelines have fair PPV for detection of AN, which would lead to avoidable resections in patients without AN. Additionally, the high-risk features of both guidelines do not accurately identify all patients with AN., (Copyright © 2016 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Bullous pemphigoid (BP) is an acquired, autoimmune, subepidermal blistering disorder. A possible paraneoplastic association has been suggested; however, debate remains regarding the precise relationship of these neoplasms with BP. We present a case of recalcitrant BP in a 67-year-old man with a pancreatic neoplasm that was found to be a lymphoepithelial cyst. Immunoperoxidase staining of the cyst demonstrated C 3 d along the basement membrane of the stratified squamous epithelium, suggesting that the BP may have involved the lymphoepithelial cyst itself. Shortly after excision of the cyst, BP rapidly resolved without any immunosuppressive treatment, raising the possibility that the immunologic process involving the lymphoepithelial cyst of the pancreas was the inciting factor for the patient's cutaneous disease. Although rare, some cases of BP may be a paraneoplastic process. A thorough screening via patient history and directed laboratory studies may be warranted in recalcitrant cases.

Pancreatic endocrine tumors (PETs) or islet cell tumors are rare lesions, the incidence of which is estimated to be less than 1 per 100,000 person-years in the general population. PETs can be divided into functional (exhibit a distinct clinical syndrome due to hormone hypersecretion) and non-functional tumors. The majority of PETs are non-functional. In spite of their rarity, cystic neoplasms of the pancreas are characterized by existing or potential malignancy that cannot be ignored during decisive process with regard to the choice of treatment. The purpose of this workis to find an association with Crohn's disease and cystic degeneration of a neuroendocrine tumor of the pancreas. Crohn's disease may affect extraintestinal organs, including the pancreas. In such cases, It seems certain that many patients diagnosed with Crohn disease (CD) are predisposed to a wider spectrum of cancers. We present a case of pancreatic cyst with no typical features of pseudocyst in the medical interview, with history of Crohn's disease, treated by caudal pancreatectomy. We tried to evaluate the clinical and morphological features of so-called cystic neoplasms associated with inflammatory bowel disease and to define their pathological characteristics., Competing Interests: The authors declare that they have no competing interests.

Unlabelled: *, Objective: Patients with potentially premalignant neoplastic pancreatic cysts without high-risk stigmata usually enter a surveillance program. Data on outcomes of such surveillance programs are scarce. We aimed to evaluate the resection rate and malignancy rate during follow-up., Material and Methods: From our prospective database (2006-2015) of patients with pancreatic cysts, we analyzed patients with pancreatic cysts without high-risk stigmata with at least six months follow-up., Results: In total, 146 patients were followed for a median of 29 months (IQR 13.5-50 months). In 124 patients (84.9%), no changes in clinical or imaging characteristics occurred during follow-up. Thirteen patients (8.9%) developed an indication for surgery after a median follow-up of 25 months (IQR 12-42 months). Two patients did not undergo surgery because of comorbidity, 11 patients (7.5%) underwent resection. Indications for surgery were symptoms (n = 2), development of a pancreatic mass (n = 1), a new nodule (n = 2), thickened cyst wall (n = 1), pancreatic duct dilation (n = 3), and/or suspicion of mucinous cystic neoplasm (MCN) (n = 3). Postoperative histology showed one pancreatic malignancy not originating from the cyst, three mixed type-intraductal papillary mucinous neoplasm (IPMN), one side branch-IPMN, two MCN, one neuroendocrine tumor, one serous cystadenoma, one inflammatory cyst, and one lymphangioma. The highest grade of cyst dysplasia was borderline dysplasia., Conclusions: Most neoplastic pancreatic cysts without high-risk stigmata at initial presentation show no substantial change during 1-4-year follow-up. Only 7.5% of patients underwent surgery and less than 1% of patients developed pancreatic malignancy. This indicates that additional markers are needed to tailor treatment of pancreatic cysts.

Although a mucinous nonneoplastic cyst (MNNC) of the pancreas is defined as a benign nonneoplastic lesion with no malignant potential, its histogenesis and etiology are still uncertain. To explore the origin and development of MNNC, we searched for neoplasia-associated mutational change in oncogene and tumor suppressor genes. Specifically, we analyzed KRAS oncogene mutations by polymerase chain reaction/dideoxy DNA (Sanger) sequencing and tumor suppression gene deletion by loss of heterozygosity (LOH) using polymerase chain reaction/capillary gel electrophoresis for a panel of 16 polymorphic microsatellite repeat markers targeting common tumor suppression gene loci at 1p, 3p, 5q, 9p, 10q, 17p, 17q, 18q, 21q, and 22q on DNA isolated from the cystic lining epithelium microdissected from 15 surgically diagnosed MNNCs by microdissection of unstained histologic sections of fixed resection specimens. DNA mutations were demonstrated in 4 of 15 cases: 1 with KRAS mutation at codon 12 glycine (G) substitution by aspartic acid (D) (G12D), 1 with KRAS mutation at 12 glycine (G) substitution by arginine (R) (G12R), 1 with LOH at 10q (PTEN), and 1 with LOH at 17q (RNF43). Therefore, although the genomic mutation rate detected in MNNC is relatively low, our results indicate that MNNCs may acquire genetic alteration similar to low-grade pancreatic intraepithelial neoplasia, furthering debate of the true nature of these lesions., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: The work-up of cystic lesions of the pancreas often involves endoscopic ultrasound (EUS) with fine needle aspiration (FNA). In addition to CEA and amylase measurement, fluid is routinely sent for cytologic examination. We evaluated the utility of cytologic findings in clinical decision-making., Materials and Methods: Records of patients who underwent EUS-guided pancreatic cyst aspiration were reviewed. Findings from axial imaging and EUS were compared to cyst fluid cytology as well as fluid amylase and CEA. All results were then compared to final diagnosis, determined by clinical analysis for those patients not resected, and surgical pathology report for those who underwent resection., Results: A total of 167 patients were reviewed. Of 48 patients with suspicious findings on imaging, cytology yielded diagnostic information in 89.6 % of cases (43 patients). However, in the 119 patients where no suspicious components were revealed on imaging, fluid cytology yielded no significant diagnostic results in any case. In all cases where mucin was noted on cytologic review, thick fluid was also seen at the time of aspiration., Discussion: In our cohort of patients with cystic pancreatic lesions, cytologic analysis of pancreatic cyst fluid yielded no diagnostic benefit over radiologic findings alone. In such cases where fluid is to be aspirated, specimens that would otherwise be sent for cytologic evaluation would be better served for other purposes, such as molecular analysis or banking for future research.

Objectives: Endoscopic ultrasonography-guided ethanol ablation therapy for pancreatic cystic lesions is a minimally invasive treatment but still is an experimental therapy. The aims were to investigate the safety and efficacy of endoscopic ultrasonography-guided ethanol ablation therapy., Methods: The inclusion criteria were the following: clinically indeterminate pancreatic cystic lesions in radiologic imaging studies, 2 to 5 cm unilocular or oligolocular cysts without communication to main pancreatic duct, and patients with high-risk operation., Results: There were 91 study patients with median follow-up of 40 months. The response rate was as follows: complete resolution, 41 (45%); partial resolution, 37; and persistent cysts, 13. Pancreatic cystic lesions were categorized based on cystic fluid analysis: 9 intraductal papillary mucinous neoplasms (IPMNs), 12 mucinous cystic neoplasms, 33 serous cystic neoplasms, and 28 uncategorized cysts. The success rate was significantly different according to cystic fluid analysis (serous cystic neoplasm, 58%; mucinous cystic neoplasm, 50%; IPMN, 11%; uncategorized cysts, 39%; P < 0.0001). There were 3 patients with mild pancreatitis after the treatment., Conclusions: Endoscopic ultrasonography-guided ethanol ablation therapy seems to be a safe treatment modality. However, it was only effective in 11% of IPMNs. Therefore, the clinical application should be very limited for certain patients who could not tolerate the surgical treatment.

Cystic neoplasms and neuroendocrine adenomas of the pancreas are detected increasingly more frequently and in up to 50 % as asymptomatic tumors. Intraductal papillary mucinous neoplasms, mucinous cystic neoplasms and solid pseudopapillary neoplasms are considered to be premalignant lesions with different rates of malignant transformation. The most frequent neuroendocrine adenomas are insulinomas. Neuroendocrine adenomas are considered to be potentially malignant, inherent to the lesion and development is unpredictable. Standard surgical treatment for pancreatic tumors are the Kausch-Whipple resection, left hemipancreatectomy and total pancreatectomy depending on the location; however, the application of standard surgical procedures, which are usually multiorgan resections for benign, premalignant and low-risk cancers of the pancreas have to be balanced against the risk for early postoperative morbidity, hospital mortality of 1.5-7 % and loss of endocrine and exocrine pancreatic functions in 12-30 %. Tumor enucleation, pancreatic middle segment resection and duodenum-preserving total pancreatic head (DPPHR-T/S) resection are parenchyma-preserving, local resection procedures, which are associated with a low early postoperative rate of severe complications, hospital mortality up to 1.3 % and maintenance of exocrine and endocrine pancreatic functions in more than 90 %. Tumor enucleation bears the risk of pancreatic fistulas (<33 %) and a limitation is proximity to the pancreatic main duct. The main risk for pancreatic middle segment resection is early postoperative pancreatic fistulas (up to 40 %), early postoperative intra-abdominal hemorrhage and a reintervention frequency up to 15 %. The DPPHR-T/S resection is applied for cystic neoplastic lesions in 90 %, severe postoperative complications are below 15 % and the 90-day hospital mortality is 0.5 %. Pancreatic fistulas are observed in less than 20 % with a recurrence rate of <1 %. These facts and maintenance of exocrine and endocrine pancreatic functions are advantages compared with the Kausch-Whipple resection of the pancreatic head. The use of tumor enucleation, particularly for neuroendocrine tumors and pancreatic middle segment resection as well as total DPPHR resection should replace the pancreatoduodenectomy for lesions in the pancreatic head and hemipancreatectomy for lesions in the pancreatic body and tail.

The detection rate of cystic lesions of the pancreas has increased following the widespread use of high-resolution imaging technologies. CT-scan, MRI and echo-endoscopy are diagnostic modalities. Pseudocyst is the most common lesion. It is benign and can be managed with endoscopic treatment. Mucinous cystic neoplasia and Intraductal Papillary Mucinous Neoplasia (IPMN) carry a risk for malignant transformation. The surgical treatment of these lesions has to be discussed by a multidisciplinary board. Serous cystic neoplasia and pseudopapillar and solid neoplasia are two rare types of lesion. The aim of this article is to present the diagnostic pathway and the management of these lesions from the general practitioner point of view.

A lymphoepithelial cyst (LEC) is a rare pancreatic lesion, histologically showing squamous epithelia, dense lymphoid tissues, and a keratin substance. Cross-section images of the pancreatic LEC typically show a well demarcated unilocular or multilocular cyst without a solid component. Here we report a rare case of pancreatic LEC in which multiple floating ball-like components were depicted via endoscopic ultrasound. The ball-like components were also depicted by various imaging methods such as computed tomography (CT) showing low-density components, T1-weighted magnetic resonance imaging (MRI) and diffusion-weighted imaging (DWI) showing high-intensity components, and T2-weighted MRI showing low-intensity components. The ball-like components in all images were not well enhanced. Laparotomic cyst resection was performed, and the surgical material revealed keratin balls inside the pancreatic LEC. Keratin components of a pancreatic LEC can take a liquid, sludge, or solid form. Clinicians must be aware of the variations in imaging to facilitate the differentiation and management of pancreatic cystic lesions.

Purpose: The purpose of this study was to retrospectively evaluate diagnostic accuracy of cystic lesions of the pancreas in order to determine if less aggressive surgical treatment might be safe and therefore warranted., Methods: A retrospective cohort study was conducted in 232 patients with either observed or resected cystic lesions of the pancreas referred for evaluation and treatment to the University Medical Center Freiburg, Germany, between 2001 and 2011., Results: Most patients had MRI or CT for preoperative imaging (90.6 %). Preoperatively, benign pseudocysts (BPC) were diagnosed in 84 (36.2 %) patients and intraductal papillary mucinous neoplasm (IPMN) in 59 (25.2 %) patients, whereas serous cyst adenoma, mucinous cystic neoplasm (MCN), solid pseudopapillary tumors (SPPTs), and neuroendocrine tumors (NETs) were less common. In 43 % of patients, the preoperative diagnosis concurred with the postoperative diagnosis. The preoperative diagnosis was accurate in BPC, less so in IPMN, and inaccurate in MCN, NET, and SPPT. However, prediction of tumor biology was accurate; only 11 % of the lesions regarded as benign turned out to be malignant after resection, and no patient without resection developed malignancy at a median follow-up of 8 months. Subsequently, 89 % of diagnosed benign tumors had indeed benign pathology., Conclusions: The prediction of biology is often correct, whereas specific diagnosis is often wrong. A considerable amount of benign lesions are treated more aggressively than warranted if malignancy is suspected prior to surgery. Parenchyma-sparing techniques might be an option, but prospective multicenter studies need to follow. Experienced pancreatic radiologists can improve accuracy of preoperative biology.

Background: Gastric duplication is a rare malformation mostly diagnosed during childhood. Symptoms in adults are atypical, rare, or may be completely absent. The diagnosis is suggested after a morphological and histological assessment. The treatment is a complete surgical resection., Case Report: We report on a case of a 28-year-old woman referred to our unit for a surgical assessment of a gastric duplication of the antropyloric area associated with paraduodenal and pancreatic extensions, diagnosed by several image tools and histological confirmation. She had undergone a total laparoscopic resection of the duplication without violation of the gastric lumen or any other splanchnic injury. The postoperative course was uneventful and the patient was discharged on postoperative day seven without any complains., Conclusions: The present report illustrates that complete resection of a distal gastric duplication is feasible by a laparoscopic minimal invasive procedure and therefore is considered to be a safe therapeutic modality. Our case is the first distal gastric duplication cyst with pancreatic and paraduodenal extension reported in the literature completely resected by laparoscopic approach.

Background/objectives: In absence of evidence-based guidelines of pancreatic cystic neoplasms (PCN), the management might vary among physicians. The aim of this survey was to assess the attitude of Dutch gastroenterologists (GE) towards the management of asymptomatic PCNs., Methods: An anonymous online questionnaire was distributed to all practicing GE (n = 381) in The Netherlands, in which four vignette patients with PCN were presented., Results: In total 45% of GE responded. Most respondents would perform surveillance for a 10 mm PCN (78%) mainly with an interval of one year (57%). A shorter interval of three (26%) or six (57%) months was chosen for a 25 mm BD-IPMN. Ultrasound was recommended for surveillance by 19% for a 10 mm cyst. GE with EUS experience were more likely to apply EUS for surveillance of 10 mm cyst than those without (56% vs 28%; p < 0.001). The presence of a branch-duct intraductal mucinous neoplasm (BD-IPMN) with a mural nodule, dilated pancreatic duct (8 mm) or increased serum CA 19.9 (300 U/ml) were considered an indication for resection by respectively 88%, 68% and 51% of respondents., Conclusion: Dutch GE demonstrate substantial variability in the management of asymptomatic PCNs. A significant proportion of general GE still use ultrasound for surveillance of small PCNs, while GE with EUS experience were more likely to perform EUS. The presence of risk factors for malignant degeneration of IPMN were not recognized by a substantial proportion of GE. Data on the natural history of PCNs is required to provide input for evidence-based guidelines, which should lead to a more uniform approach., (Copyright © 2016 IAP and EPC. Published by Elsevier B.V. All rights reserved.)

Background: Management of cystic lesions of the pancreas (CLP) is controversial. In this study, we sought to evaluate national changes in the resection of CLP over time, to better understand the impact of evolving guidelines on CLP management., Methods: We used Medicare data to examine CLP resection among patients undergoing pancreatic resection between 2001 and 2012. Patients with a diagnosis of CLP were identified and compared to patients with non-CLP indications. We then examined changes over time in patient and hospital characteristics and outcomes among patients with a CLP diagnosis., Results: We identified 56,419 Medicare patients undergoing pancreatic resection, of which 2129 had a CLP diagnosis. The annual number of CLP resections, and proportion of all resections performed for CLP increased significantly during the period, from 2.1% (65/3072) resections in 2001, to 4.5% (286/6348) in 2012 (p < 0.001). The proportion of CLP resections with a malignant diagnosis did not change (15.5% in 2001-2003 vs. 13.1% in 2010-2012, p = 0.4). Overall rates of 30-day mortality decreased significantly during the period (9.6% in 2001-2003 vs. 5.5% in 2010-2012, p < 0.001)., Discussion: CLP resections were performed with increasing frequency in Medicare patients between 2001 and 2012, but this did not correspond to increased diagnosis of malignancy. Additional research is needed to understand the influence of recent guidelines on management of CLP., (Copyright © 2016 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Macrocystic serous cystadenoma is an unusual and essentially benign pancreatic tumor. Herein, we report on a 40-year-old woman diagnosed with macrocystic serous cystadenoma who presented with obstructive jaundice. A cystic lesion in the head and body of the pancreas was revealed by abdominal computed tomography. Intraoperative pancreatic cyst aspiration ruled out mucinous cystic neoplasm which has a malignant potential. The pancreatic cyst fluid cytology was basophilic amorph materials concluded as benign cystic lesion. Internal drainage was performed instead of pancreatic resection which showed good outcome. Biliary obstruction is a rare complication of serous cystadenoma. This case describes an unusual clinical presentation of macrocystic serous cystadenoma.

Background: Cytology plays a pivotal role in the preoperative diagnosis of pancreatic cysts. Here the Papanicolaou Society of Cytopathology (Pap Society) guidelines were used to reclassify and assess the malignancy risk of cytology diagnoses of histologically proven pancreatic neoplastic mucinous cysts., Methods: A database search (January 2000 to June 2014) was performed for pancreatic neoplastic mucinous cyst resections with endoscopic ultrasound-guided fine-needle aspiration within the preceding year. Histologic diagnoses were reclassified according to the 2010 Word Health Organization criteria. For atypical/suspicious/positive cytology diagnoses, the cytology slides were reviewed, blinded to the histologic diagnoses. The cysts were reclassified according to the Pap Society guidelines, and the findings were correlated with the histology., Results: One hundred thirty-eight cases of pancreatic neoplastic mucinous cysts were retrieved. Eleven cases with atypical/suspicious cytology diagnoses with unavailable slides were excluded. The remaining 127 cases included 81 intraductal papillary mucinous neoplasms and 46 mucinous cystic neoplasms. The sensitivity of cytology for the diagnosis of neoplastic mucinous cysts was 76.4%. The sensitivity, specificity, and accuracy of cytology for the diagnosis of malignancy (high-grade dysplasia or worse) were 48.3%, 94.9%, and 84.3%, respectively. The risk of malignancy was 17.4% for the nondiagnostic category, 0% for the negative category, 13% for the neoplastic category, 63.6% for the atypical category, 80% for the suspicious category, and 100% for a positive diagnosis., Conclusions: This study reveals that the Pap Society guidelines allow the accurate categorization of pancreatic neoplastic mucinous cysts with cytology. The diagnostic categories (from negative to positive) are associated with an increasing risk of malignancy, and this can further aid in patient management and risk stratification., (© 2015 American Cancer Society.)

Management of cystic neoplasms of the pancreas is challenging as it relies on radiologic and cyst fluid markers to discriminate between benign and pre-cancerous lesions, however their ability to predict malignancy is limited. While asymptomatic serous cystadenomas can be managed conservatively, mucinous cystic neoplasms and intraductal papillary mucinous neoplasms are more difficult to manage. A selective approach, based on the preoperative likelihood of high-grade dysplasia or invasive disease, is the standard of care. Research is focusing on the development of pre-operative markers for identifying high risk lesions, which will spare patients with low-risk or benign lesions the risks of pancreatectomy., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Organ-sparing pancreatic resection is important in prophylactic surgery for cystic neoplasms. There is controversy regarding the optimal surgical approach for pancreatic lesions in the neck or proximal body of the pancreas. Central compared with distal pancreatectomy is technically more challenging, but preserves more functional pancreatic tissue. Because of the prophylactic nature of the surgery and long survival of patients with benign and borderline malignant lesions, surgeons need to stratify greater importance to surgical morbidity and sparing pancreatic parenchyma., Patient: The patient is a 59-year-old active woman with a symptomatic cystic neoplasm of the pancreas exhibiting high-risk imaging features. The cyst of 2.2 × 1.8 cm in the body of the pancreas was impinging on the portal venous confluence., Technique: The patient was positioned in the French Position, the lesser sac was opened, and the pancreatic body exposed. A retropancreatic tunnel was created with staple division of the neck. The body was mobilized off the portal vein and splenic vessels transected. A retrogastric pancreaticogastrostomy was sewn through an anterior gastrotomy. The stent was delivered past the pylorus to decrease pancreatic enzymatic activation. Pathology demonstrated a mixed predominantly branch duct IPMN with multifocal high grade dysplasia and PanIN3., Conclusions: Laparoscopic ultrasound helps in defining cyst borders, and minimal blood loss optimizes visualization during the dissection. A minimally invasive pancreaticogastrostomy created through an anterior gastrotomy is technically feasible and safe. This approach can minimize the morbidity of prophylactic pancreatic surgery for patients with cystic neoplasms. Nevertheless, it should not compromise safety, oncologic completeness, or an organ-sparing approach.

The approach to incidentally noted pancreatic cysts is constantly evolving. While surgical resection is indicated for malignant or higher risk cysts, correctly identifying these highest risk pancreatic cystic lesions remains difficult. Using parameters including cyst size, presence of solid components, and pancreatic duct involvement, the 2012 International Association of Pancreatology (IAP) and the 2015 American Gastroenterological Association (AGA) guidelines have sought to identify the higher risk patients who would benefit from further evaluation using endoscopic ultrasound (EUS). Not only can EUS help further assess the presence of solid component and nodules, but also fine needle aspiration of cyst fluid aids in diagnosis by obtaining cellular, molecular, and genetic data. The impact of new endoscopic innovations with novel methods of direct visualization including confocal endomicroscopy require further validation. This review also highlights the differences between the 2012 IAP and 2015 AGA guidelines, which include the thresholds for sending patients for EUS and surgery and methods, interval, and duration of surveillance for unresected cysts.

Retrospective analysis of 108 observation files of 66 patients, operated on for chronic pancreatitis complications and 42 — for postnecrotic pancreatic cysts, using draining, resection—draining and resection operations, was conducted. Morphological changes in pancreatic parenchyma and pancreatic postnecrotic cysts at the operation time were compared with intraoperative blood loss, rate and character of intraoperative and post/ operative complications, depending on the kind, duration, volume and adequacy of the operations performed, as well as with lethality rate. Disadvantages in surgical treatment were noted in 57 (57%) patients, and 4 (3.7%) patients died. Unsuccessful surgical treatment in 64.9% observations was caused by incapacity to perform an adequate surgical intervention, the anastomosis sutures insufficiency, postoperative hemorrhage and significant intraoperative blood loss

An analysis of treatment results was made in 26 patients with postnecrotic cysts of different degree of maturity. The laparotomy with trans-gastral cystogastrostomy on the external drainage were performed on 11 patients. A new modified operation underwent 15 patients. This operation was transgastric cystogastrostomy on the external drainage from mini-access, which was developed in 2011. The laparotomy of 4–5 cm was made in pseudocyst projection of the pancreas using a standard set of tools «mini-assistant». Postoperative period have passed without complications in all the patients. The drainage was removed on 30–40 days of postoperative period. The terms of hospital stay reduced from (14,4±0,9) bed/ days to (10,8±0,5) bed/days due to application of proposed method of treatment.

Epidermoid cysts arising from both the pancreas and spleen are rare. We herein report a case of a surgically resected epidermoid cyst in the pancreatic tail invaginated from the spleen. A multi-locular cyst, 2 cm in diameter, without a solid component was discovered incidentally in the pancreatic tail. During the 11-year follow-up, the emergence of satellite cystic lesions with distinct appearances was seen, and surgical resection was selected despite the lack of any associated symptoms or evidence of cytological abnormalities. Histologically, these cysts were lined with benign multi-layered flattened epithelium surrounded by a thin layer consisting of cells positive for CD8 and CD68 and connecting to the spleen.

Objective: Endoscopic ultrasound (EUS) plays an integral role in the evaluation of pancreatic cysts lesions (PCLs). The aim of the study was to determine predictors of surgical referral in patients with PCLs undergoing EUS., Methods: We performed a multicenter retrospective study of patients undergoing EUS for evaluation of PCLs. Demographics, EUS characteristics, and fine-needle aspiration results were recorded. Patients were categorized into surgery or surveillance groups on the basis of post-EUS recommendations. Univariate and multivariate analyses were performed to identify predictors of surgical referral., Results: 1804 patients were included. 1301 patients were recommended to undergo surveillance and 503 patients were referred for surgical evaluation, of which 360 patients underwent surgery. Multivariate analysis revealed the following 5 independent predictors of surgical referral: symptoms of weight loss on presentation (odds ratio [OR], 2.69; 95% confidence interval [CI], 1.44-5.03), EUS findings of associated solid mass (OR, 7.34; 95% CI, 3.81-14.16), main duct communication (OR, 4.13; 95% CI, 1.71-9.98), multilocular macrocystic morphology (OR, 2.79; 95% CI, 1.78-4.38), and fine-needle aspiration findings of mucin on cytology (OR, 3.06; 95% CI, 1.94-4.82)., Conclusions: This study identifies factors associated with surgical referral in patients with PCLs undergoing EUS. Future studies should focus on creation of risk stratification models to determine the need for surgery or enrollment in surveillance programs.

This is a case of a newborn female with congenital pancreatic cysts discovered incidentally. The 5-week-old infant had multiple abdominal cysts originating from the pancreas. When the radiologist catheter placement failed to alleviate the symptoms, the infant underwent laparoscopic excision. The lesion, however, recurred 11 months after the first excision, leading to a second surgical procedure including excision and marsupialization. A review of the literature revealed that this is a rare condition. Herein, we discuss the characteristics of the case, including medical imaging, drainage catheter placement, surgical treatment, pathological findings, and follow-up. Differential diagnoses, clinical presentations, treatment options, and patient outcomes are also discussed. Although rare, congenital pancreatic cyst should be considered in the differential diagnosis of an infant with cystic lesion of the pancreas.

Background/aims: Pancreatic cystic lesions have a broad spectrum of differential diagnosis. There is an ongoing demand to identify specific and sensitive cystic fluid markers for the differential diagnosis of pancreatic cysts. We aimed to evaluate the diagnostic value of cystic fluid chromogranin A (CgA) in the differential diagnosis of pancreatic cysts., Materials and Methods: Patients who underwent endoscopic ultrasound (EUS)-guided aspiration for pancreatic cysts were included in the study. Cytopathological analysis and biochemical analysis, including cystic fluid carcinoembryonic antigen (CEA), amylase, Ca 19-9, and CgA, were performed., Results: Fifty-three patients were included in the study. The final diagnosis of patients was 14 pancreatic pseudocysts, 10 intraductal papillary mucinous neoplasms (IPMNs), 8 mucinous cystic neoplasms (MCN), 8 serous cystadenomas (SCAs), 2 cystic pancreatic neuroendocrine tumors (PNETs), and 11 others. The mean CgA levels were 50.51±130.04 ng/mL in pseudocysts, 12.38±8.59 in MCN, and 13.76±10.90 in cystic PNET. There was only one patient with a very high cystic fluid CgA (515.49 ng/mL) and was diagnosed as pseudocyst developed in chronic pancreatitis patient. Two patients with cystic PNET had normal levels of cystic fluid CgA., Conclusion: Cystic fluid CgA is not a useful marker for the differential diagnosis of cystic PNETs. It also has no value in the differential diagnosis of other pancreatic cysts.

Background & Aims: The management of pancreatic cysts poses challenges to both patients and their physicians. We investigated whether a combination of molecular markers and clinical information could improve the classification of pancreatic cysts and management of patients., Methods: We performed a multi-center, retrospective study of 130 patients with resected pancreatic cystic neoplasms (12 serous cystadenomas, 10 solid pseudopapillary neoplasms, 12 mucinous cystic neoplasms, and 96 intraductal papillary mucinous neoplasms). Cyst fluid was analyzed to identify subtle mutations in genes known to be mutated in pancreatic cysts (BRAF, CDKN2A, CTNNB1, GNAS, KRAS, NRAS, PIK3CA, RNF43, SMAD4, TP53, and VHL); to identify loss of heterozygozity at CDKN2A, RNF43, SMAD4, TP53, and VHL tumor suppressor loci; and to identify aneuploidy. The analyses were performed using specialized technologies for implementing and interpreting massively parallel sequencing data acquisition. An algorithm was used to select markers that could classify cyst type and grade. The accuracy of the molecular markers was compared with that of clinical markers and a combination of molecular and clinical markers., Results: We identified molecular markers and clinical features that classified cyst type with 90%-100% sensitivity and 92%-98% specificity. The molecular marker panel correctly identified 67 of the 74 patients who did not require surgery and could, therefore, reduce the number of unnecessary operations by 91%., Conclusions: We identified a panel of molecular markers and clinical features that show promise for the accurate classification of cystic neoplasms of the pancreas and identification of cysts that require surgery., (Copyright © 2015 AGA Institute. Published by Elsevier Inc. All rights reserved.)

Cystic neoplasms of the pancreas are found with increasing prevalence, especially in elderly asymptomatic individuals. Although the overall risk of malignancy is very low, the presence of these pancreatic cysts is associated with a large degree of anxiety and further medical investigation due to concerns about malignancy. This review discusses the different cystic neoplasms of the pancreas and reports diagnostic strategies based on clinical features and imaging data. Surgical and nonsurgical management of the most common cystic neoplasms, based on the recently revised Sendai guidelines, is also discussed, with special reference to intraductal papillary mucinous neoplasm (IPMN; particularly the branch duct variant), which is the lesion most frequently identified incidentally. IPMN pathology, its risk for development into pancreatic ductal adenocarcinoma, the pros and cons of current guidelines for management, and the potential role of endoscopic ultrasound in determining cancer risk are discussed. Finally, surgical treatment, strategies for surveillance of pancreatic cysts, and possible future directions are discussed.

This update critiques the key abstracts relevant to endoscopic evaluation or interventions in pancreatic and biliary diseases that were presented at Digestive Disease Week (DDW) 2015. We selected 20 abstracts based on their clinical significance, relevance to endoscopic practice and impact on patient management., (© 2015 The Authors Digestive Endoscopy © 2015 Japan Gastroenterological Endoscopy Society.)

Pancreatic cancer continues to have an extremely poor prognosis. There have been hardly any therapeutic advances in the last few years and consequently attention is focussed on early diagnosis. In this regard, endoscopic ultrasonography and several associated techniques, such as electrography or the use of intravenous contrast agents, continue to be the cornerstone of differential diagnosis. In the latest Digestive Diseases Week, numerous presentations were made on cystic pancreatic tumours, especially intraductal papillary mucinous tumours, with their well-known potential for malignant transformation. In addition to the problems of the preoperative characterization of these entities, by both endoscopic ultrasound cytological evaluation--even with the presence of an on-site pathologist--and by intracystic markers, the role of other techniques was also mentioned, such as confocal laser endomicroscopy or the use of intravenous contrast agents to characterize the wall nodule. There were numerous studies on the natural history of intraductal papillary mucinous tumours, which mainly supported the increasingly conservative approach adopted by the recent Fukuoka international guidelines. Certain aspects were highlighted, such as comorbidities, when considering surgery, or the growth rate of the tumour. In treatment, endoscopic ultrasound-guided injection of gemcitabine and paclitaxel, without the need for alcohol as an ablative treatment of mucinous cystic tumours, is gaining ground in specific cases., (Copyright © 2015 Elsevier España, S.L.U. All rights reserved.)