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Objective: To quantify the rate of progression in surveilled cysts and assess what factors should indicate delayed resection., Background: Side-branch intraductal papillary mucinous neoplasms (SB-IPMNs) are increasingly discovered, making it challenging to identify which patients require resection, thus avoiding inappropriate treatment. Most incidental lesions are surveyed, yet the consequences of that decision remain uncertain., Methods: A prospectively maintained database of pancreatic cystic neoplasms was queried for patients with SB-IPMN. Patients with ≥2 imaging studies >6 months apart were included. Clinically relevant progression (CR-progression) was defined by symptoms, worrisome/high-risk stigmata, or invasive cancer (IC). Growth ≥5 mm in 2 years is considered CR-progression; size ≥3 cm alone is not., Results: Between 1997 and 2023, 1337 patients were diagnosed with SB-IPMN. Thirty-seven (2.7%) underwent up-front surgery; 1000 (75.0%) had >6 months of surveillance.The rate of CR-progression was 15.3% (n = 153) based on size increase (n = 63, 6.3%), main-duct involvement (n = 48, 4.8%), symptoms (n = 8, 5.0%), or other criteria (n = 34, 3.4%). At a median follow-up of 6.6 years (interquartile range: 3.0-10.26), 17 patients (1.7%) developed IC. Those with CR-progression developed IC in 11.1% (n = 17) and high-grade dysplasia (HGD) in 6.5% (n = 10). Nearly half of the cancers were not contiguous with the surveyed SB-IPMN.Size ≥3 cm was not associated with HGD/IC ( P = 0.232). HGD/IC was least common in CR-progression determined by size growth (6.3%) versus main-duct involvement (24%) or other (43%, P < 0.001)Patients with CR-progression demonstrated improved survival (overall survival) with resection on time-to-event ( P < 0.001) and multivariate Cox regression (hazard ratio = 0.205, 0.096-0.439, P < 0.001) analyses. Overall survival was not improved with resection in all patients ( P = 0.244)., Conclusions: CR-progression for SB-IPMNs is uncommon, with the development of cancer anywhere in the pancreas being rare. Initial size should not drive resection. Long-term and consistent nonoperative surveillance is warranted, with surgery currently reserved for CR-progression, knowing that the majority of these still harbor low-grade pathology., Competing Interests: The authors report no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To identify risk factors associated with the progression of pancreatic cysts in patients undergoing surveillance., Background: Previous studies of intraductal papillary mucinous neoplasms (IPMNs) rely on surgical series to determine malignancy risk and have inconsistently identified characteristics associated with IPMN progression., Methods: We conducted a retrospective review of 2197 patients presenting with imaging concerning for IPMN from 2010 to 2019 at a single institution. Cyst progression was defined as resection or pancreatic cancer development., Results: The median follow-up time was 84 months from the presentation. The median age was 66 years, and 62% were female. Ten percent had a first-degree relative with pancreatic cancer, and 3.2% had a germline mutation or genetic syndrome associated with an increased risk of pancreatic ductal adenocarcinoma (PDAC). Cumulative incidence of progression was 17.8% and 20.0% at 12 and 60 months postpresentation, respectively. Surgical pathology for 417 resected cases showed noninvasive IPMN in 39% of cases and PDAC with or without associated IPMN in 20%. Only 18 patients developed PDAC after 6 months of surveillance (0.8%). On multivariable analysis, symptomatic disease [hazard ratio (HR)=1.58; 95% CI: 1.25-2.01], current smoker status (HR=1.58; 95% CI: 1.16-2.15), cyst size (HR=1.26; 95% CI: 1.20-1.33), main duct dilation (HR=3.17; 95% CI: 2.44-4.11), and solid components (HR=1.89; 95% CI: 1.34-2.66) were associated with progression., Conclusions: Worrisome features on imaging at presentation, current smoker status, and symptomatic presentation are associated with IPMN progression. Most patients progressed within the first year of presentation to Memorial Sloan Kettering Cancer Center (MSKCC). Further investigation is necessary to develop personalized cyst surveillance strategies., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: Prospective evaluation of 2 clinical-molecular models in patients with unknown pathology who underwent endoscopic ultrasound with fine-needle aspiration (EUS-FNA) for a cystic lesion of the pancreas., Summary of Background Data: Preoperative prediction of histologic subtype (mucinous vs nonmucinous) and grade of dysplasia in patients with pancreatic cystic neoplasms is challenging. Our group has previously published 2 clinical-molecular nomograms for intraductal papillary mucinous neoplasms (IPMN) that incorporated both clinical/radiographic features and cyst fluid protein markers (sFASL, CA72-4, MMP9, IL-4)., Methods: This multiinstitutional study enrolled patients who underwent EUS-FNA for a cystic lesion of the pancreas. Treatment recommendations regarding resection were based on standard clinical, radiographic, and endoscopic features. Predicted probabilities of high-risk IPMN (high-grade dysplasia/invasive cancer) were calculated using the previously developed clinical-molecular nomograms., Results: Cyst fluid was obtained from 100 patients who underwent diagnostic EUS-FNA. Within this group there were 35 patients who underwent resection, and 65 were monitored radiographically. Within the group that underwent resection, 26 had low-risk IPMN or benign non-IPMN lesions, and 9 had high-risk IPMN. Within the surveillance group, no patient progressed to resection or developed cancer after a median follow-up of 12months (range: 0.5-38). Using the clinical/radiographic nomogram alone, 2 out of 9 patients with high-risk IPMN had a predicted probability >0.5. In the clinical-molecular models, 6 of 9 patients in model 1, and 6 of 9 in model 2, had scores >0.5., Conclusions: This prospective study of patients with unknown cyst pathology further demonstrates the importance of cyst fluid protein analysis in the preoperative identification of patients with high-risk IPMN. Longer follow-up is necessary to determine if this model will be useful in clinical practice., Competing Interests: The authors report no conflicts of interest, (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: There is a paucity of literature evaluating new-onset diabetes mellitus (NODM) after resection of pancreatic cystic lesions (PCLs). We sought to characterize the incidence and risk factors associated with NODM after partial pancreatectomy for PCLs., Methods: We utilized the IBM MarketScan Database (2012-2018) to identify all nondiabetic adults who underwent partial pancreatectomy for PCLs. Patients with any other pancreatic disease were excluded. We performed Kaplan-Meier analysis and multivariable Cox proportional hazards regression to define the incidence and risk factors of postoperative NODM., Results: Among 311 patients, the overall risk (95% confidence interval) of NODM was 9.1% (6.3-12.9%), 15.1% (11.3-20.2%), and 20.2% (15.3-26.4%) at 6, 12 and 24 months, respectively. Multivariable analysis (adjusted hazard ratio; 95% confidence interval) revealed that older age (1.97; 1.04-3.72; 55-64 vs 18-54 years), obesity (2.63; 1.35-5.12), hypertension (1.79; 1.01-3.17), and cardiovascular disease (2.54; 1.02-6.28) were independent predictors of NODM. Rates of NODM were similar after distal pancreatectomy versus pancreaticoduodenectomy., Conclusions: Within 2 years, 1 in 5 patients without any other pancreatic disease will develop NODM after partial pancreatectomy for PCLs. Those with advanced age, metabolic syndrome features, and/or cardiovascular disease may benefit from preoperative counseling and intensive postoperative monitoring, education, and treatment for diabetes mellitus., Competing Interests: The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Center for Advancing Translational Sciences or the National Institutes of Health. No other potential conflicts of interest exist for any author in relationship to this publication., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Mucinous cystic neoplasms and solid pseudopapillary neoplasms are the most common pancreatic tumors occurring in women of fertile age and in pregnant women. The aim of this study is to provide an updated literature review on this association and to present a fully laparoscopic resection of a pregnancy-associated pancreatic cystic neoplasm., Materials and Methods: A systematic literature review was performed using PubMed (MEDLINE), Scopus, Ovid, ISI Web of Science, and Google Scholar for searching. The syntax was (pancr*) AND (cyst*) AND (pregn*) AND (tumor). Only English-language articles describing pancreatic surgical resections were included., Results: Forty-seven case reports were included. The mean age of the patients was 29.6±5.3. Nine patients (20%) required emergency surgery, 4 (9%) due to cyst rupture, and 5 (11%) due to hemorrhage. Four patients (9%) suffered a miscarriage, and 2 (5%) opted for pregnancy termination; the rest of the women delivered a healthy newborn (86%, n=36). Thirty percent (n=14) of the resected neoplasms were malignant, and among mucinous cystic lesions, this raised to 45% (n=11). All patients diagnosed during the third trimester were resected postpartum, whereas 26/34 (76%) of patients diagnosed during the first 2 trimesters underwent surgery before delivery., Conclusions: The most worrisome complications in pregnancy-associated pancreatic cysts are bleeding or rupture. Mucinous cystic neoplasm has a tendency to grow during pregnancy. A postpartum resection was generally preferred when the cystic neoplasm was diagnosed during the third trimester. This report is the first to describe a fully laparoscopic pancreatic resection., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Rationale: Mucinous cystic neoplasms (MCNs) are pancreatic mucin-producing cystic lesions with a distinctive ovarian-type stroma. The diagnosis is generally easy in typical cases; however, differential diagnosis is difficult in others such as in the case we report herein., Patient Concerns: A 27-year-old woman with sudden onset of epigastric pain was referred to our hospital for suspected acute pancreatitis. Contrast-enhanced computed tomography revealed a 25-mm cystic lesion in the pancreas and a low density area with delayed enhancement at the right upper side of the cystic lesion., Diagnoses: During its clinical course, the cystic lesion underwent various morphological changes. Eventually, it presented typical findings of MCNs, and could be accurately diagnosed., Interventions: Laparoscopic distal pancreatectomy was performed on the patient by preserving the spleen., Outcomes: The patient revealed no symptoms till 1 year after the operation., Lessons: This case of MCN with intriguing short-term morphological changes was associated with recurrent pancreatitis. A combination of imaging modalities is essential for accurate diagnosis of MCNs, and follow-up with serial imaging might be useful for certain unusual lesions.

Purpose of Review: With recent advances in radiographic testing, there have been increases in the incidental identification of pancreatic cysts. Determining management, specifically, which cysts can be watched versus intervened upon has significant impact on at the patient-physician level as well as globally on resource allocation and healthcare expenditures. This review focuses on the three main guidelines (Fukuoka guidelines, European consensus, and American Gastroenterological Association recommendations) for management of pancreatic cystic disease after incidental identification., Recent Findings: There are iterant revisions to each set of recommendations every few years as new data is published on the subject. This review encompasses the most up-to-date recommendations for management while also providing a framework for conceptualizing work-up for varying types of pancreatic cystic disease before and after incidental identification., Summary: Pancreatic cystic disease should be approached in thoughtful clinical manner, and each cyst should be evaluated in the context of patient's health and healthcare goals. All cysts, however, should be considered malignant until proven otherwise. Applications of different diagnostic modalities like MRI and EUS with FNA can better characterize cystic lesions and assess malignant potential. Resection should always be considered in cysts with concerning features. If deferred, surveillance should be continued indefinitely. Early termination of surveillance risks future malignant progression.

Asymptomatic pancreatic cysts are a common clinical problem but only a minority of these cases progress to cancer. Our aim was to compare the accuracy to detect malignancy of the 2015 American Gastroenterological Association (AGA), the 2012 International Consensus/Fukuoka (Fukuoka guidelines [FG]), and the 2010 American College of Radiology (ACR) guidelines.We conducted a retrospective study at 3 referral centers for all patients who underwent resection for an asymptomatic pancreatic cyst between January 2008 and December 2013. We compared the accuracy of 3 guidelines in predicting high-grade dysplasia (HGD) or cancer in resected cysts. We performed logistic regression analyses to examine the association between cyst features and risk of HGD or cancer.A total of 269 patients met inclusion criteria. A total of 228 (84.8%) had a benign diagnosis or low-grade dysplasia on surgical pathology, and 41 patients (15.2%) had either HGD (n = 14) or invasive cancer (n = 27). Of the 41 patients with HGD or cancer on resection, only 3 patients would have met the AGA guideline's indications for resection based on the preoperative cyst characteristics, whereas 30/41 patients would have met the FG criteria for resection and 22/41 patients met the ACR criteria. The sensitivity, specificity, positive predictive value, negative predictive value of HGD, and/or cancer of the AGA guidelines were 7.3%, 88.2%, 10%, and 84.1%, compared to 73.2%, 45.6%, 19.5%, and 90.4% for the FG and 53.7%, 61%, 19.8%, and 88% for the ACR guidelines. In multivariable analysis, cyst size >3 cm, compared to ≤3 cm, (odds ratio [OR] = 2.08, 95% confidence interval [CI] = 1.11, 4.2) and each year increase in age (OR = 1.07, 95% CI = 1.03, 1.11) were positively associated with risk of HGD or cancer on resection.In patients with asymptomatic branch duct-intraductal papillary mucinous neoplasms or mucinous cystic neoplasms who underwent resection, the prevalence rate of HGD or cancer was 15.2%. Using the 2015 AGA criteria for resection would have missed 92.6% of patients with HGD or cancer. The more "inclusive" FG and ACR had a higher sensitivity for HGD or cancer but lower specificity. Given the current deficiencies of these guidelines, it will be important to determine the acceptable rate of false-positives in order to prevent a single true-positive.

Objective: In 2015, the American Gastroenterological Association recommended the discontinuation of radiographic surveillance after 5 years for patients with stable pancreatic cysts. The current study evaluated the yield of continued surveillance of pancreatic cysts up to and after 5 years of follow up., Methods: A prospectively maintained registry of patients evaluated for pancreatic cysts was queried (1995-2016). Patients who initially underwent radiographic surveillance were divided into those with <5 years and ≥5 years of follow up. Analyses for the presence of cyst growth (>5 mm increase in diameter), cross-over to resection, and development of carcinoma were performed., Results: A total of 3024 patients were identified, with 2472 (82%) undergoing initial surveillance. The ≥5 year group (n = 596) experienced a greater frequency of cyst growth (44% vs. 20%; P < 0.0001), a lower rate of cross-over to resection (8% vs 11%; P = 0.02), and a similar frequency of progression to carcinoma (2% vs 3%; P = 0.07) compared with the <5 year group (n = 1876). Within the ≥5 year group, 412 patients (69%) had demonstrated radiographic stability at the 5-year time point. This subgroup, when compared with the <5 year group, experienced similar rates of cyst growth (19% vs. 20%; P= 0.95) and lower rates of cross-over to resection (5% vs 11%; P< 0.0001) and development of carcinoma (1% vs 3%; P= 0.008). The observed rate of developing cancer in the group that was stable at the 5-year time point was 31.3 per 100,000 per year, whereas the expected national age-adjusted incidence rate for this same group was 7.04 per 100,000 per year., Conclusion: Cyst size stability at the 5-year time point did not preclude future growth, cross-over to resection, or carcinoma development. Patients who were stable at 5 years had a nearly 3-fold higher risk of developing cancer compared with the general population and should continue long-term surveillance.

Pancreatic cysts are commonly found on cross-sectional imaging. The question arises in determining which lesions are premalignant or malignant and may require further testing, intervention, or follow-up. In pancreatic cysts without obvious malignancy on imaging, we approach them using the Four "S" Criteria. These are (1) symptoms that may be originating from the pancreatic cyst; (2) size of the cyst 2 cm or larger and/or main pancreatic duct greater than 5 mm; (3) survival of the patient, based on comorbidity index to determine surgical fitness; and then endoscopic ultrasound with fine needle aspiration (FNA) recommended to determine (4) solid component presence in the cyst, namely, nodule or thick walls, as well as to perform FNA to obtain cyst content. Current cyst fluid analysis options include use of cytology to determine presence of malignancy and carcinoembryonic antigen and fluid genetics to identify potentially premalignant lesions. The aims of this article are to explore current management guidelines for pancreatic cysts, present a comprehensive approach to pancreatic cysts, and explain the advantages and disadvantages of each option for evaluation of pancreatic cysts including endoscopic ultrasound with FNA with cyst fluid analysis using an evidence-based approach.

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.

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.

Background: Pancreatic fluid collections can form after episodes of pancreatitis, either acute or chronic. The majority will resolve spontaneously but when decompression is mandated, endoscopic drainage is the method of choice. However, it is not void of complications., Methods: We retrospectively reviewed the charts of 65 patients who underwent endoscopic drainage of pancreatic fluid collections in our institution. The primary outcomes examined included the incidence and type of complications associated with the endoscopic approach., Results: Endoscopic ultrasound was utilized in 86.2% and transgastric approach was used in 81.5% of the cases. The complication rate was 17%. Specifically, complications recorded were infection (6%), perforation and acute abdomen necessitating surgical intervention (4.6%), pneumoperitoneum that was managed nonoperatively (3%), upper gastrointestinal bleed in the knife puncture site that resolved spontaneously (1.5%), and stent migration (1.5%). One patient died remotely to the endoscopic drainage after paracentesis of ascites that resulted in hemorrhagic shock., Conclusions: This study is one of the largest studies reporting the associated morbidity and mortality after endoscopic cyst-gastrostomy. Major and minor complications occurred at a rate of 17% in our study. Endoscopic approach is a safe draining method and should remain the approach of choice for pancreatic fluid collection decompression.

Objectives: Lymphoepithelial cyst (LEC) of the pancreas is an unusual and benign cystic tumor. Accurate preoperative diagnosis is difficult; hence, most of pancreatic LECs are resected. The aim was to describe clinicopathological features of pancreatic LEC to guide appropriate management., Methods: We retrospectively collected data about LEC patients treated in our department between 1987 and 2012 and added cases from review of the literature during the same period., Results: One hundred seventeen cases (3 from our institution and 114 from literature review) were identified. Most patients were men (78%). The discovery was generally fortuitous. Serum CA19-9 was elevated in half of the cases. No specific radiological feature was identified. Fine needle aspiration and cytologic analysis allowed a correct preoperative diagnosis in 21% of the patients, showing presence of squamous cells, lymphocytes, and keratinous debris. Half of them were treated conservatively, whereas other patients underwent surgery. Neither malignant transformation nor recurrence after resection was observed., Conclusions: The LEC of the pancreas is a rare benign tumor that could be treated conservatively. Fine needle aspiration is the only tool that can achieve a diagnosis without resection. If no certain diagnosis can be made, surgery is warranted to rule out a malignant differential diagnosis.

Objectives: The objectives of this study were to assess the long-term quality of life (QOL) after the resection of a primary pancreatic cyst and to determine predictors of outcome. Secondary outcomes were pancreatic function and survival., Methods: One hundred eight consecutive patients, who underwent resection between 1992 and 2007 and had nearly 60 months follow-up, were reviewed. Questionnaires and function tests were collected during scheduled outpatient clinic visits., Results: At follow-up, 20 patients had died. Five-year overall survival was 94% for benign and 62% for malignant neoplasia. Of 88 living patients, 65 (74%) returned questionnaires. Generic physical and mental QOL scores were equal or better compared with healthy references. None of the disease-specific symptom scales were above mean 50, implicating none to mild complaints. Independent predictors for good generic QOL were young age (P < 0.05) and resected malignancy (P < 0.05); predictors for good gastrointestinal QOL were male sex (P < 0.1), limited resection (P < 0.05), endocrine insufficiency (P < 0.05), and employment (P < 0.05). Endocrine insufficiency prevalence was 40%, and 59% for exocrine insufficiency., Conclusions: After cyst resection, long-term QOL is equal to healthy references, pancreatic insufficiency is prevalent but does not impair QOL, and survival relates positive compared with solid pancreatic adenocarcinoma. The excellent long-term outcome justifies proceeding with surgery once a medical indication for resection has been established.

Objective: The aim of this study was to critically analyze the safety of the revised guidelines, with focus on cyst size and worrisome features in the management of BD-IPMN., Background: The Sendai guidelines for management of branch duct (BD) intraductal papillary mucinous neoplasm (IPMN) espouse safety of observation of asymptomatic cysts smaller than 3 cm without nodules (Sendai negative). Revised international consensus guidelines published in 2012 suggest a still more conservative approach, even for lesions of 3 cm or larger. By contrast, 2 recent studies have challenged the safety of both guidelines, describing invasive carcinoma or carcinoma in situ in 67% of BD-IPMN smaller than 3 cm and in 25% of "Sendai-negative" BD-IPMN., Methods and Results: Review of a prospective database identified 563 patients with BD-IPMN. A total of 240 patients underwent surgical resection (152 at the time of diagnosis and 88 after being initially followed); the remaining 323 have been managed by observation with median follow-up of 60 months. No patient developed unresectable BD-IPMN carcinoma during follow-up. Invasive cancer arising in BD-IPMN was found in 23 patients of the entire cohort (4%), and an additional 21 patients (3.7%) had or developed concurrent pancreatic ductal adenocarcinoma. According to the revised guidelines, 76% of resected BD-IPMN with carcinoma in situ and 95% of resected BD-IPMN with invasive cancer had high-risk stigmata or worrisome features. The risk of high-grade dysplasia in nonworrisome lesions smaller than 3 cm was 6.5%, but when the threshold was raised to greater than 3 cm, it was 8.8%, and 1 case of invasive carcinoma was found., Conclusions: Expectant management of BD-IPMN following the old guidelines is safe, whereas caution is advised for larger lesions, even in the absence of worrisome features.

Purpose of Review: An increasing number of patients are being diagnosed with pancreatic cysts. Pancreatic cysts are best evaluated by a team of healthcare professionals that includes gastroenterologists, surgeons, radiologists, pathologists, oncologists and geneticists., Recent Findings: The international consensus guidelines for the management of intraductal papillary mucinous neoplasm (IPMN) and mucinous cystic neoplasm were updated in 2012, incorporating research that had been reported over a 5-year span since the publication of the previous guidelines. There are significant changes in the new guidelines, which include redefining main duct IPMN and removing the recommendation for surgical resection based on size alone. In addition, the discovery of molecular markers of cyst type promises to revolutionize the way patients are diagnosed and managed., Summary: The diagnosis and management of patients with pancreatic cysts have progressed significantly in recent years. Large prospective, multicenter trials are now needed to validate the new international consensus guidelines and to assess the accuracy of new molecular markers.

Objectives: An undercharacterized subclass of pancreatic mucinous cysts without histologic characteristics of neoplasia is emerging. This article aimed to highlight the clinical characteristics and implications of this new subset of pancreatic cystic lesions., Methods: The clinical, radiologic, and pathologic features of all cysts that underwent operative resection at a tertiary referral pancreatic disease center from February 2005 to June 2009 were reviewed. Immunohistochemistry for mucinous peptide antigens was selectively performed., Results: Of 104 operations, a pathologic examination revealed 52 intraductal papillary mucinous neoplasms, 9 mucinous cystadenomas, 17 serous cystadenomas, 9 pseudocysts, 5 solid pseudopapillary tumors, 2 carcinomas, 1 cystic pancreatic endocrine tumor, and 2 other cystic lesions. Seven mucinous cysts without neoplastic features were identified, representing 2% of all pancreatic resections, 6.7% of all resected cysts, and 10.3% of the 68 mucinous cysts. There was no evidence of cytologic atypia, papillary growth, or ovarian-type stroma in any of the cases. MUC1, MUC2, and MUC5AC were expressed in 83%, 0%, and 100%, respectively. There has been no recurrence with a mean follow-up of 44 months., Conclusions: This underappreciated entity belongs to the family of mucinous pancreatic cysts. However, unifying clinical characteristics that would prevent unnecessary resections in patients harboring these seemingly benign lesions are currently lacking.

Objectives: The aim of the study was to assess the diagnosis and management of solid pancreatic neoplasm in children and the type of surgical treatment, focusing on short- and long-term outcomes., Methods: We retrospectively reviewed the charts of all children who had undergone pancreatic resection for suspicion of pancreatic tumor in Kremlin Bicêtre Hospital, Paris, between 1986 and 2008. We studied the symptoms at diagnosis, the type of surgery, and the short- and long-term morbidity and mortality., Results: Of 18 patients identified, there were 7 pseudopapillary tumors, 3 neuroblastomas, 2 rhabdomyosarcomas, 1 acinar cell carcinoma, 1 endocrine cell carcinoma, 1 renal angiomyolipoma, and 3 pancreatic cysts. Symptoms at diagnosis were abdominal trauma, abdominal mass, and jaundice. Operative procedures were duodenopancreatectomy (11), mid-pancreatic resections (2), splenopancreatectomy (2), distal pancreatectomy (1), and tumorectomy (2). There were no deaths related to surgery. The postoperative morbidity rate was 45%, including 2 cases of fistula (11%) occurring after a mid-pancreatic resection and a pancreaticoduodenectomy. The median follow-up was 4.2 years (range 2-11). There was no diabetes mellitus, but there was 1 case of fat diet intolerance requiring pancreatic enzyme substitution. All of the children had a growth curve within normal limits., Conclusions: In this experience, pancreatic resections have proven to be a safe and efficient procedure, with low long-term morbidity, for the treatment of tumoral and selected nontumoral pancreatic masses.

Lymphoepithelial cysts (LECs) of the pancreas are rare true pancreatic cysts. Their cause is not known. The differential diagnosis is broad and includes many benign and malignant cystic lesions of the pancreas and surrounding organs. A combination of imaging modalities and fine needle aspiration might narrow the differential diagnosis. However, the final diagnosis can only be achieved with certainty after resection of the cyst. In this study, we report the largest LEC of the pancreas to have been resected laparoscopically. A 43-year-old man presented with upper abdominal pain, a 7.5 cm mutlioculated cystic mass in the pancreatic body and tail on imaging, and a raised serum cancer antigen-19-9. Laparoscopic distal pancreatectomy and splenectomy was performed. Histologic examination revealed a LEC. This study discusses the diagnostic difficulties and management decisions which face surgeons treating pancreatic cysts.

Purpose of Review: Pancreatic cystic neoplasms are increasingly identified and their management remains uncertain. Recent studies demonstrate an evolving clinical approach., Recent Findings: The vast majority of asymptomatic pancreatic cysts without concerning clinical or imaging features can be observed without surgery. Clinical predictors for malignancy at surgery include male sex, age above 50 years, weight loss, and high cyst fluid carcinoembryonic antigen (CEA), but these factors are insufficient for patient selection. Endoscopic ultrasound (EUS)-guided fine needle aspiration with cyst fluid analysis for risk stratification and selective resection appears the most cost-effective approach. In addition to CEA, DNA analysis, differential protein expression, and proteomic studies of cyst fluid may be helpful in differentiating cystic lesions in selected patients. EUS-guided ethanol lavage of cysts resulted in regression; this method may have a role in treatment in the future. More future research investigating the safety of this procedure, technique modifications, and choice of agent is needed., Summary: The approach to incidentally discover pancreatic cystic lesions is challenging due to the difficulty in preoperative definitive lesion characterization. Recently developed diagnostic and treatment strategies show promise for improved patient outcomes.

Beckwith-Wiedemann syndrome is associated with an increased risk of tumors in the pediatric age. We report the case of a newborn with Beckwith-Wiedemann syndrome presenting the simultaneous occurrence of a cystic pancreatoblastoma and an adrenal neuroblastoma. Surgery was required to treat the pancreatoblastoma, and a "wait and see" policy was adopted for the neuroblastoma, which spontaneously regressed within a few months.

Objectives: In branch duct intraductal papillary mucinous neoplasm (IPMN) of the pancreas, the importance of the cyst size to predict malignancy is still controversial. Our aim was to elucidate the malignant potential of branch duct IPMN without mural nodules (flat branch duct IPMN)., Methods: Seventy-three patients with flat branch duct IPMNs were studied in our institution., Results: There were 6 malignant IPMNs in this series, all of which were 30 mm or more in size, whereas there was no malignancy in IPMNs of less than 30 mm. Statistically significant predictors of malignancy were atypical cytological condition and main pancreatic duct (MPD) diameter of 5 mm or more. The cyst size of 30 mm or more tended to be associated with malignancy. The frequency of malignancy in flat branch duct IPMNs with the size of 30 mm or more and MPD diameter of less than 5 mm was 3.6%, whereas there were 5 malignant cases (26.3%) in flat branch duct IPMNs with the size of 30 mm or more and MPD diameter of 5 mm or more., Conclusions: We conclude that the size criteria (> or =30 mm) to predict malignancy proposed in the international consensus guidelines is appropriate and resection or meticulous follow-up using cytological examination and MPD dilatation is needed in patients with flat branch duct IPMNs.

Objectives: Pancreatic cysts are being found with increasing frequency. Although symptomatic cysts should be resected, what to do about asymptomatic cysts is less clear. The purpose of this study was to determine threshold values to choose resection over observation for a patient with an asymptomatic pancreatic cyst., Methods: Decision analysis assesses the consequences of a decision based on occurrence probabilities. This decision analysis assessed 1 decision, to resect or observe a patient with an asymptomatic pancreatic cyst. The consequences for resection are operative mortality/no operative mortality, short-term morbidity of patients surviving the operation, benign/malignant cyst, and life expectancy after resection of malignant lesions. The probabilities are based on the extant literature. The age-specific life expectancy for benign cysts was considered the same as for the general age-specific population from the 2002 United States Life Table. Because the operative mortality, distribution of benign versus malignant cysts, and life expectancy after resection or observation for malignant cysts vary, sensitivity analysis was done to assess the threshold values of these factors when resection becomes favored over observation., Results: The baseline decision analysis is based on the following assumptions: 30% of lesions are malignant, operative mortality rate is 3%, and the 5-year survival rate of resected malignant cysts is 50%. Varying on age, the risk of the cystic lesion being malignant to favor resection is greater than 11.6% to 15.5%, the operative mortality rate has to be less than 7.4% to 13.8%, and the life expectancy gain by resecting, rather than observing a malignant lesion, has to be greater than 3.49 to 5.38 years., Conclusions: The decision to resect must be based on the surgeon's operative mortality rate, the predicted operative mortality for the individual patient, the probability that the lesion is malignant, and the survival difference between resecting and observing malignant cystic lesions. Overall, for resection to be recommended, the physician must believe that the risk that the lesion is malignant is approximately 15% or greater, that the life expectancy gained from resection be approximately 5 years or greater, and that the surgeons' operative mortality rate be approximately 8% or less.

Purpose of Review: New understanding of the dynamic of acute pancreatitis, the clinical impact of local pathology in chronic pancreatitis and cystic neoplastic lesions bearing high potential for malignant transformation has changed the management of pancreatic diseases., Recent Findings: In acute pancreatitis, risk factors independently determining outcome in severe acute pancreatitis are early and persistent multiorgan failure, infected necrosis and extended sterile necrosis. The management of severe acute pancreatitis is based on early intensive-care treatment and late surgical debridement. In chronic pancreatitis, recent data from randomized controlled clinical trials have demonstrated duodenum-preserving pancreatic head resection with an inflammatory mass of the head as superior to pylorus-preserving Whipple resection. Cystic neoplasms are local lesions of the pancreas with high malignant potential. Local organ-preserving resection techniques have been applied with low morbidity and mortality, replacing a Whipple-type resection. Resection of pancreatic cancer is ineffective to cure patients. After an R0-resection, a significant survival benefit has been achieved when adjuvant chemotherapy has additionally been applied., Summary: New knowledge about the nature of inflammatory diseases, cystic neoplastic lesions and malignant pancreatic tumours has changed the indication for surgical treatment and the application of organ-preserving surgical techniques.

Objective: To define a group of patients with pancreatic cysts who do not require resection., Summary Background Data: The increased use of cross-sectional imaging has resulted in an increased identification of small, asymptomatic pancreatic cysts. Data have not been available to determine which lesions should be resected., Methods: All patients evaluated at our institution between January 1995 and January 2005 for the ICD-9 diagnosis of pancreatic cyst were reviewed. Analysis was performed to identify associations between patient and cyst characteristics, and selection of operative or nonoperative management., Results: Pancreatic cysts were evaluated in 539 patients. Initial management was operative in 170 patients (32%), and nonoperative (radiographic follow-up) in 369 patients (68%). Factors associated with initial operative management included presence of a solid component (45% vs. 6%, P < 0.001), larger size of the lesion (mean 4.8 cm vs. 2.4 cm, P = 0.001), and presence of symptoms (44% vs. 16%, P = 0.001). Malignancy was present in 18% (32 of 170) of patients initially resected. Mucinous tumors (n = 18) were the most common malignant histologic subtype. None of the invasive cancers arising from mucinous cysts was <3 cm. Median radiographic follow-up in patients initially managed nonoperatively was 24 months (range, 1-172 months). In 29 patients (8%), changes developed within the cyst that resulted in resection; malignancy was present in 11 of 39 (38%), representing 3% (11 of 369) of all patients being followed radiographically., Conclusions: Selected patients with cystic lesions <3 cm in diameter and without a solid component may be followed radiographically with a malignancy risk (3% this study) that approximates the risk of mortality from resection. Malignancy within mucinous tumors is associated with size, and small mucinous tumors are very unlikely to be malignant.

Heterotopic pancreas is normal pancreatic tissue located in an abnormal location. Heterotopic pancreas in the esophagus is a rare congenital anomaly. We present a unique case of pancreatitis and retention cyst in a heterotopic pancreas in the esophagus, outline the investigation and management of cystic lesions of the distal esophagus, and review the literature.

Objectives: To describe the frequency and range of pancreatic disorders in children requiring surgical intervention and to highlight the importance of multidisciplinary management., Methods: An audit of all children under 17 years of age referred with surgical disorders of the pancreas or pancreatitis to a regional pediatric gastroenterology unit in the United Kingdom during a 10-year period. A retrospective chart review of clinical features, pathology and outcome was undertaken., Results: Surgical intervention was required for the following pancreatic disorders: persistent hyperinsulinemic hypoglycemia of infancy (n = 4), pancreatic tumors (n = 5), pancreaticobiliary malunion (n = 12), pancreatic trauma (n = 6) and pancreatitis (n = 10). The indications for surgery in acute pancreatitis were a persistent pseudocyst (n = 1) and treatment of an underlying cause of pancreatitis (n = 4); in chronic pancreatitis, surgery was used to treat symptomatic pancreatic duct strictures (n = 4). One child died of a progressive lymphoma but all others who underwent surgery are alive and well. All 33 children with acute pancreatitis, including four with pancreatic necrosis, survived., Conclusions: Surgery for pancreatic disorders in children is rarely required but may be necessary a) for definitive management of primary pancreatic pathology, b) to treat sequelae of acute or chronic pancreatitis and c) to treat an underlying cause of pancreatitis. There is a broad spectrum of potential pathologies. These patients are best managed by a multidisciplinary team approach.

Advances in minimally invasive surgery have revolutionized the field of surgery. Despite the great strides in equipment and experience, operative conduct remains confined by the limits of exposure. Retroperitoneal fat can be abundant and can contribute greatly to difficulty in exposure. Visceral organs ventral to the retroperitoneum preclude direct access and require optimal patient positioning to operate. Additionally, the major vascular pedicles all originate in the retroperitoneum off of the abdominal aorta or enter the inferior vena cava. The pancreas, in particular, is surrounded by the portal vein, celiac axis, superior mesenteric vein and artery, and splenic vein and artery. If injured during surgery, these vessels can present a life-threatening emergency. The issues related to the vasculature, coupled with the difficulty in resecting portions of the pancreas and the relative paucity of pancreatic procedures, have greatly concentrated these cases at tertiary care centers staffed by experienced laparoscopists. However, as surgical technology improves and fellowships train more surgeons with advanced laparoscopic skills, minimally invasive pancreatic surgery may diffuse with more community-based health care networks.

Objective: The objectives of this analysis were to define the incidence, natural history, and predictors of neoplasia in pancreatic cysts to determine which patients can safely be observed and which should undergo an operation., Summary Background Data: With advancements in imaging technology, cystic lesions of the pancreas are being detected with increased frequency. Many of these lesions are small and asymptomatic, but they may be associated with pancreatitis or have malignant potential. Therefore, the management of these patients is complex, and knowledge of pancreatic cyst natural history and predictors of neoplasia are important., Methods: From January 1995 through December 2002, all radiologic, surgical, and pathology records were reviewed for the presence of pancreatic cysts. In determining natural history, only patients with 2 scans more than 1 month apart at our institution were included. Patients with a clinical history and laboratory evidence of pancreatitis and/or pathologic confirmation of a pseudocyst were excluded. Factors analyzed as potential predictors of neoplasia included age, gender, cyst size, and symptoms. Serous cystadenomas, solid and cystic papillary (Hamoudi) tumors, lymphoepithelial cysts and simple cysts were all benign, whereas mucinous cystic neoplasms, intraductal papillary mucinous neoplasm, cystic neuroendocrine tumors, and cystadenocarcinomas were considered to be premalignant or malignant., Results: Among 24,039 CT or MR scans, 290 patients (1.2%) had pancreatic cysts, and 168 of these patients (0.7%) had no documentation of pancreatitis. Seventy-nine of these patients with 103 cysts had more than 1 scan with an average interval of 16 months. These cysts increased in size in 19%, did not change in 59% and decreased in 22% of patients. Forty-nine patients underwent surgery for 14 benign (serous cystadenomas = 10, Hamoudi = 2, lymphoepithelial = 1, simple = 1) 25 premalignant (mucinous cystic neoplasm =16, intraductal papillary mucinous neoplasm = 5, neuroendocrine tumors = 4), or 10 malignant (intraductal papillary mucinous neoplasm = 7, cystadenocarcinomas = 3) lesions. Gender and cyst size did not predict neoplasia. However, presence of symptoms predicted premalignant or malignant pathology (60% vs. 23%, P < 0.05), and age over 70 years was associated with malignancy (60% vs. 21%, P < 0.02)., Conclusions: These data suggest that cystic pancreatic neoplasms 1) occur in 0.7% of patients, 2) increase in 19% over 16 months, and 3) are likely (60%) to be malignant in patients older than 70 years. Therefore, we recommend surgical excision for pancreatic cysts that are increasing under observation, symptomatic, or detected radiologically in fit older patients.

Introduction and Aims: Cystic neoplasms of the pancreas may be inadvertently treated as benign pseudocysts in clinical practice, often without the use of cytology, cyst tumor markers, or histopathology. We assessed the utility of EUS-guided fine-needle aspiration (EUS-FNA) to assist in the diagnosis and management of pancreatic cysts., Methodology: All patients who had pancreatic cysts detected by EUS over a 24-month period were analyzed. Preoperative diagnosis was derived from an algorithm combining clinical history and endosonographic features. In selected cases, EUS-FNA was performed and cyst fluid aspirates were analyzed. Surgical specimens served as diagnostic standard., Results: A total of 43 patients with pancreatic cysts underwent 45 EUS examinations. Surgical specimens were obtained from 9 patients (mucinous cystadenocarcinoma, 3; adenocarcinoma, 3; pancreatic endocrine tumor, 2; and benign cyst, 1); diagnostic EUS correctly predicted malignant cysts in 8/9 (88.9%). One case inaccurately interpreted by EUS as cystic neoplasm turned out to be a benign cyst on resection. Twenty-one patients underwent EUS-FNA. The cytologic interpretation was adenocarcinoma in 9.5% (2/21); suspicious for malignancy or atypical cells in 19.0% (4/21); benign in 66.6% (14/21); and insufficient cells in 4.8% (1/21)., Conclusion: The information gathered from clinical history and EUS, complemented by fluid analysis after EUS-guided FNA, predicts neoplastic pancreatic cysts and assists in decision-making for medical or surgical approach.

There are few reports about magnetic resonance cholangiopancreatography (MRCP) in cystic lesions of the pancreas (KLP). For this reason, we have undertaken a prospective study evaluating the diagnostic efficiency of MRCP as compared with ultrasonography. Twenty-four patients with KLP were examined with magnetic resonance imaging (MRI), and standard and cholangiopancreatography. There were seven cases of cysts associated with acute pancreatitis, 11 patients with KLP and chronic calcifying pancreatitis, five cases of cystic neoplasms of the pancreas, and one polycystic disease of the gland. All cases were first submitted to ultrasonography, which failed to diagnose only a case of cyst associated with chronic pancreatitis depicted by MRCP. We used a GE Signa Horizon 1.5-T system (20 examinations) and a Siemens Magneton Plus 1.5-T machine (four examinations). Eleven patients were operated on. In all cases, it was possible to identify the cysts, the main pancreatic duct and the biliary tree, and verify the relationship of the cyst with neighboring organs. Communication of the cyst with the main pancreatic duct was described in five instances, but we cannot be sure that MRCP would have depicted all cyst-duct communications. The MRI and MRCP images were confirmed by surgery in the 11 operated-on cases. The diagnosis of duct alterations and small pancreatic stones in initial cases of chronic calcifying pancreatitis may be problematic. Clinical findings are very important data to be considered in the differential diagnosis of KLP. Together with the clinical data, MRCP is a very important technique in the diagnostic and therapeutic decision making of KLP. Standard magnetic resonance is advisable as part of the examination in all cases. MRCP is not invasive, is reliable if one knows its limitations, and the patient can return at once to his activities. It allows the analysis of many variables in one examination, contributing to better therapy.

Endoscopic pancreatography was useful in demonstrating pancreatic ductal obstruction, fistula, and pseudocyst in the preoperative evaluation of a patient with pancreatic ascites. Surgical exploration confirmed the preoperative findings and Roux-en-Y cystojejunostomy was performed. Some salient features of pancreatic ascites are discussed.

Of 92 patients with moderately severe acute pancreatitis initially studied within three weeks of onset by ultrasonic tomography, 52 developed an acute fluid collection in the lesser sac. Documentation of the ultrasound prediction of pseudocyst was achieved by surgery or autopsy in 26 cases. Spontaneous resolution of the acute pseudocyst was demonstrated by serial ultrasonography and radiogrphy in another 10 patients. Exploration exposed 3 false positive predictions of pseudocyst. Eleven other patients with a cystic configuration either refused surgery or were lost to followup. Acute pseudocyst formation is a relatively common phenomenon in the early phases of moderately severe pancreatitis. While spontaneous resolution of acute pseudocysts is frequent, in approximately 50% of cases acute pseudocysts progress to chronic pseudocysts. A distinction between acute and chronic pseudocyst is necessary since specific surgical management depends upon the phase of pseudocyst development. Unless regional sepsis supervens, acute pseudocyts of less than three weeks' duration may be followed by serial ultrasonography in the hope of spontaneous resolution. When a pseudocyst has achieved chronic status, spontaneous resolution is rare. Persistent conservative management under these conditions invites the excessive mortality and morbidity of spontaneous rupture.

Cystoduodenostomy for internal drainage of pancreatic pseudocysts has been both condemned and condoned. The current study reports the largest personal experience with a single method of cystoduodenostomy. Transduodenal cystoduodenostomy was performed in 14 cases followed an average of 39 months without mortality, fistula formation, abscess, or hemorrhage. Recurrent pseudocysts developed in two patients, neither in the pancreatic head. In a 15-year combined review of surgical procedures for pancreatic pseudocyst, transduodenal cystoduodenostomy evidenced mortality rates similar to other forms of internal drainage. In direct contrast, laterolateral cystoduodenostomy by suture anastomosis resulted in a 70% mortality rate and should rarely, if ever, be performed.

This is the first reported case of mumps hemorrhagic pancreatitis in a child, documented at operation, and by rising mumps titers and complicated by a pseudocyst. In severe cases when surgery is indicated, drainage of the lesser sac usually prevents pseudocyst formation. If a pseudocyst occurs, drainage is required to eliminate disabling pain and chronic pancreatitis. Live attenuated mumps vaccine may eliminate this source of pancreatitis from American children in the future.