Mucins in neoplasms of pancreas,ampulla of Vater and biliary system

Tumors of the pancreas,the ampulla of Vater,and the extrahepatic and intrahepatic bile ducts have significant histological similarities due to the common embryonic origin of the pancreatobiliary system.This obviates the need for discovery of biomarkers with diagnostic and prognostic value for these tumors.Mucins,especially MUC-1,-2,-4 and-5AC,are important candidates for developing into such reliable biomarkers.Increased expression of MUC1 occurs in pancreatic ductal adenocarcinomas and is associated with increased degrees of dysplasia in pancreatic intraepithelial neoplasia(Pan IN).Positive expression of MUC2 in intraductal papillary mucinus neoplasms(IPMN) of the intestinal type indicates high potential progression to invasive carcinoma with de novo expression of MUC1,while absence of MUC2 expression in IPMNs of gastric type implies low potential to malignant evolution.De novo MUC4 expression correlates to the severity of dysplasia in Pan IN and is associated with a poor prognosis in patients with pancreatic ductal adenocarcinomas.In biliary intraepithelial neoplasia(Bil IN),increased expression of MUC1 is associated with higher degrees of dysplasia.Intrahepatic cholangiocarcinomas(ICC) are characterized by increased expression of all glycoforms of MUC1.Positive MUC2 expression in intraductal papillary neoplasm of the bile ducts(IPNB) of the intestinal type indicates high malignant potential with de novo expression of MUC1 in the invasive element.Absent MUC2 expression in any degree of Bil IN may prove useful in differentiating them from IPNB.De novo expression of MUC4 is associated with poor prognosis in patients with ICC or carcinoma of the extrahepatic bile ducts(EHBDC).High de novo expression of MUC5 AC is found in all degrees of Bil IN and all types of IPNB and ICC.The MUC5 AC is useful in the detection of neoplastic lesions of the bile duct at an early stage.Increased expression of mucin MUC1 in carcinoma of the ampulla of Vater associated with unfavorable behavior of the tumor,such as lymph node metastasis,infiltration of the pancreas and duodenum,advanced TNM classification and worse prognosis.Patients withintra-ampullary papillary-tubular neoplasm(IAPN) of the pancreatobiliary immunophenotype did not show MUC2,while those of the intestinal immunophenotype are MUC2 positive.The expression of MUC4 is associated with poor prognosis in patients with carcinoma of the ampulla of Vater favoring metastasis and making them resistant to apoptosis.Moreover,it appears that MUC4 positivity correlates with recurrence of the tumor.Expression of MUC5 AC is associated with the invasive potential of the tumor.

Comparison between solid pseudopapillary neoplasms of the pancreas and pancreatic ductal adenocarcinoma with cystic changes using computed tomography

BACKGROUND Solid pseudopapillary neoplasms of the pancreas(SPN)share similar imaging findings with pancreatic ductal adenocarcinoma with cystic changes(PDAC with cystic changes),which may result in unnecessary surgery.AIM To investigate the value of computed tomography(CT)in differentiation of SPN from PDAC with cystic changes.METHODS This study retrospectively analyzed the clinical and imaging findings of 32 patients diagnosed with SPN and 14 patients diagnosed with PDAC exhibiting cystic changes,confirmed through pathological diagnosis.Quantitative and qualitative analysis was performed,including assessment of age,sex,tumor size,shape,margin,density,enhancement pattern,CT values of tumors,CT contrast enhancement ratios,“floating cloud sign,”calcification,main pancreatic duct dilatation,pancreatic atrophy,and peripancreatic invasion or distal metastasis.Multivariate logistic regression analysis was used to identify relevant features to differentiate between SPN and PDAC with cystic changes,and receiver operating characteristic curves were obtained to evaluate the diagnostic performance of each variable and their combination.RESULTS When compared to PDAC with cystic changes,SPN had a lower age(32 years vs 64 years,P<0.05)and a slightly larger size(5.41 cm vs 3.90 cm,P<0.05).SPN had a higher frequency of“floating cloud sign”and peripancreatic invasion or distal metastasis than PDAC with cystic changes(both P<0.05).No significant difference was found with respect to sex,tumor location,shape,margin,density,main pancreatic duct dilatation,calcification,pancreatic atrophy,enhancement pattern,CT values of tumors,or CT contrast enhancement ratios between the two groups(all P>0.05).The area under the receiver operating characteristic curve of the combination was 0.833(95%confidence interval:0.708-0.957)with 78.6%sensitivity,81.3%specificity,and 80.4%accuracy in differentiation of SPN from PDAC with cystic changes.CONCLUSION A larger tumor size,“floating cloud sign,”and peripancreatic invasion or distal metastasis are useful CT imaging features that are more common in SPN and may help discriminate SPN from PDAC with cystic changes.

Diagnosis and management of relapsing pancreatitis associated with cystic neoplasms of the pancreas

One of the most important causes of relapsing pancreatitis is a cystic neoplasm of the pancreas. These low grade malignancies may cause pancreatitis by obstructing or communicating with a pancreatic duct. Patients with relapsing pancreatitis and a focal fluid fluid collection should be investigated for the possibility of a mucinous cystic neoplasm. Cross sectional imaging can provide a diagnosis with the imaging findings of a low attenuation cystic lesion containing mural calcification (CT scanning) or a lobular T2 enhancing lesion (MRCP). Endoscopic ultrasound can provide more detailed imaging with the ability to guide fine needle aspiration of the cyst fluid. Cyst fluid analysis can provide a diagnosis of a mucinous cystic lesion with the combination of cytology (mucinous epithelium), elevated carcinoembryonic antigen (CEA), and the presence of DNA mutations. Management of these patients consists of surgical resection and monitoring in patients not able to withstand surgery.

Less common neoplasms of the pancreas

Recently, there has been an increased recognition of neoplasms of the pancreas other than ductal adenocarcinorna. Although not as well studied or characterized as pancreatic adenocarcinorna there are many distinct lesions which exhibit diverse biological behaviors and varying degrees of malignancy. These lesions include： endocrine neoplasms, cystic tumors, solid pseudopapillary tumors, acinar cell carcinoma, squamous cell carcinoma, primary lymphoma of the pancreas, and metastatic lesions to the pancreas. These less common neoplasms are being diagnosed more frequently as the number and sensitivity of diagnostic imaging studies increase. This review article discusses the clinical course, diagnosis, and treatment of these less common, but quite relevant, neoplasms of the pancreas.

Imaging considerations in intraductal papillary mucinous neoplasms of the pancreas

With the widespread use of cross-sectional imaging,particularly computed tomography(CT) and magnetic resonance imaging(MRI),and the continuous improvement in the image quality of these techniques,the diagnosis of incidental pancreatic cysts has increased dramatically in the last decades.While the vast majority of these cysts are not clinically relevant,a small percentage of them will evolve into an invasive malignant tumor making their management challenging.Mucinous cystic neoplasms and intraductal papillary mucinous neoplasms(IPMN) are the most common pancreatic cystic lesions with malignant potential.Imaging findings on CT and MRI correlate tightly with the presence of malignant degeneration in these neoplasms.IPMN can be classified based on their distribution as main duct,branch duct or mixed type lesions.MRI is superior to CT in demonstrating the communication of a branch duct IPMN with the main pancreatic duct(MPD).Most branch duct lesions are benign whereas tumors involving the MPD are frequently associated with malignancy.The presence of solid nodules,thick enhancing walls and/or septae,a wide(> 1 cm) connection of a side-branch lesion with the MPD and the size of the tumor > 3 cm are indicative of malignancy in a branch and mixed type IPMN.A main pancreatic duct > 6 mm,a mural nodule > 3 mm and an abnormal attenuating area in the adjacent pancreatic parenchyma on CT correlates with malignant disease in main duct and mixed type IPMN.An accurate characterization of these neoplasms by imaging is thus crucial for selecting the best management options.In this article,we review the imaging findings of IPMN including imaging predictors of malignancy and surgical resectability.We also discuss follow-up strategies for patients with surgically resected IPMN and patients with incidental pancreatic cysts.

Differences between main-duct and branch-duct intraductal papillary mucinous neoplasms of the pancreas

In the last decade,intraductal papillary mucinous neoplasms(IPMNs) have become commonly diagnosed.From a morphological standpoint,they are classified in main-duct IPMNs(MD-IPMNs) and branch-duct IPMNs(BD-IPMNs),depending on the type of involvement of the pancreatic ductal system by the neoplasm.Despite the fact that our understanding of their natural history is still incomplete,recent data indicate that MD-IPMNs and BD-IPMNs show significant differences in terms of biological behaviour with MD-IPMNs at higher risk of malignant degeneration.In the present paper,clinical and epidemiological characteristics,rates of malignancy and the natural history of MD-IPMNs and BD-IPMNs are analyzed.The profile of IPMNs involving both the main pancreatic duct and its side branches(combined-IPMNs) are also discussed.Finally,general recommendations for management based on these differences are given.

Outcomes of nonresected main-duct intraductal papillary mucinous neoplasms of the pancreas

AIM:To compare characteristics and outcomes of resected and nonresected main-duct and mixed intraductal papillary mucinous neoplasms of the pancreas(IPMN).METHODS:Over a 14-year period,50 patients who did not undergo surgery for resectable main-duct or mixed IPMN,for reasons of precluding comorbidities,age and/or refusal,were compared with 74 patients who underwent resection to assess differences in rates of survival,recurrence/occurrence of malignancy,and prognostic factors.All study participants had dilatation of the main pancreatic duct by ≥ 5 mm,with or without dilatation of the branch ducts.Some of the nonsurgical patients showed evidence of mucus upon perendoscopic retrograde cholangiopancreatography or endoscopic ultrasound and/or after fine needle aspiration.For the surgical patients,pathologic analysis of resected specimens confirmed a diagnosis of IPMN with involvement of the main pancreatic duct or of both branch ducts as well as the main pancreatic duct.Clinical and biologic follow-ups were conducted for all patients at least annually,through hospitalization or consultation every six months during the first year of follow-up,together with abdominal imaging analysis(magnetic resonance cholangiopancreatography or computed tomography) and,if necessary,endoscopic ultrasound with or without fine needle aspiration.RESULTS:The overall five-year survival rate of patients who underwent resection was significantly greater than that for the nonsurgical patients(74% vs 58%; P =0.019).The parameters of age(< 70 years) and absence of a nodule were associated with better survival(P < 0.05); however,the parameters of main pancreatic duct diameter > 10 mm,branch ductdiameter > 30 mm,or presence of extra pancreatic cancers did not significantly influence the prognosis.In the nonsurgical patients,pancreatic malignancy occurred in 36% of cases within a mean time of 33 mo(median:29 mo; range:8-141 mo).Comparison of the nonsurgical patients who experienced disease progression with those who did not progress showed no significant differences in age,sex,symptoms,subtype of IPMN,or follow-up period; only the size of the main pancreatic duct was significantly different between these two sub-groups,with the nonsurgical patients who experienced progression showing a greater diameter at the time of diagnosis(> 10 mm).CONCLUSION:Patients unfit for surgery have a 36% greater risk of developing pancreatic malignancy of the main-duct or mixed IPMN within a median of 2.5 years.

Prognosis of invasive intraductal papillary mucinous neoplasms of the pancreas

Intraductal papillary mucinous neoplasms(IPMN) are mucin producing cystic neoplasms of the pancreas histologically classified as having non-invasive and invasive components.The five-year survival rates for non-invasive and associated invasive carcinoma are 90% and 40%,respectively in resected IPMN lesions.Invasive carcinoma within IPMN lesions can be further classified by histological subtype into colloid carcinoma and tubular carcinoma.Estimated five-year survival rates following resection of colloid carcinoma range from 57%-83% and estimated five-year survival following resection of tubular carcinoma range from 24%-55%.The difference in survival outcome between invasive colloid and tubular IPMN appears to be a function of disease biology,as patients with the tubular subtype tend to have larger tumors with a propensity for metastasis to regional lymph nodes.When matched to resected conventional pancreatic adenocarcinoma lesions by the Memorial Sloan Kettering Cancer Center pancreatic adenocarcinoma nomogram,the colloid carcinoma histological subtype has an improved estimated five-year survival outcome compared to conventional pancreatic adenocarcinoma,87% and 23%(P = 0.0001),respectively.Resected lesions with the tubular carcinoma subtype overall have a similar five-year survival outcome compared to conventional pancreatic adenocarcinoma.However,when these groups were stratified by regional lymph node status patients with negative regional lymph nodes and the tubular subtype experienced significantly better survival than patients with a similar nodal status and ductal adenocarcinoma with estimated five-year survival rates of 73% and 27%(P = 0.01),respectively.

Imaging features of intraductal papillary mucinous neoplasms of the pancreas in multi-detector row computed tomography

AIM:To retrospectively evaluate the imaging features of pancreatic intraductal papillary mucinous neoplasms (IPMNs) in multi-detector row computed tomography (MDCT).METHODS: A total of 20 patients with pathologically-confirmed intraductal papillary mucinous neoplasms (IPMNs) were included in this study. Axial MDCT images combined with CT angiography (CTA) and multiplanar volume reformations (MPVR) or curved reformations (CR) were preoperatively acquired. Two radiologists (Tan L and Wang DB) reviewed all the images in consensus using an interactive picture archiving and communication system. The disputes in readings were resolved through consultation with a third experienced radiologist (Chen KM). Finally, the findings and diagnoses were compared with the pathologic results.RESULTS: The pathological study revealed 12 malignant IPMNs and eight benign IPMNs. The diameters of the cystic lesions and main pancreatic ducts (MPDs) were significantly larger in malignant IPMNs compared with those of the benign IPMNs (P<0.05). The combined-type IPMNs had a higher rate of malignancy than the other two types of IPMNs (P<0.05). Tumors with mural nodules and thick septa had a significantly higher incidence of malignancy than tumors without these features (P<0.05). Communication of side-branch IPMNs with the MPD was present in nine cases at pathologic examination. Seven of them were identified from CTA and MPVR or CR images. From comparison with the pathological diagnosis, the sensitivity, specificity, and accuracy of MDCT in characterizing the malignancy of IPMN of the pancreas were determined to be 100%, 87.5% and 95%, respectively.CONCLUSION: MDCT with CTA and MPVR or CR techniques can elucidate the imaging features of IPMNs and help predict the malignancy of these tumors.

Large-duct pattern invasive adenocarcinoma of the pancreas–a variant mimicking pancreatic cystic neoplasms: A minireview

Pancreatic cancer currently has no subtypes that inform clinical decisions;hence,there exists an opportunity to rearrange the morphological and molecular taxonomy that guides a better understanding of tumor characteristics.Nonetheless,accumulating studies to date have revealed the large-duct type variant,a unique subtype of pancreatic ductal adenocarcinoma(PDA)with cystic features.This subtype often radiographically mimics intraductal papillary mucinous neoplasms(IPMNs)and involves multiple small cysts occasionally associated with solid masses.The“bunch-of-grapes”sign,an imaging characteristic of IPMNs,is absent in large-duct PDA.Large-duct PDA defines the mucin profile,and genetic alterations are useful in distinguishing large-duct PDA from IPMNs.Histologically,neoplastic ducts measure over 0.5 mm,forming large ductal elements.Similar to classic PDAs,this subtype is frequently accompanied by perineural invasion and abundant desmoplastic reactions,and KRAS mutations in codon 12 are nearly ubiquitous.Despite such morphological similarities with IPMNs,the prognosis of large-duct PDA is equivalent to that of classic PDA.Differential diagnosis is therefore essential.

Expression of the tumor suppressor gene maspin and its significance in intraductal papillary mucinous neoplasms of the pancreas

BACKGROUND: Maspin is a member of the serpin family of protease inhibitors and is thought to inhibit carcinoma invasion, metastasis, and angiogenesis and induce apoptosis. We examined maspin expression immunohistochemically and assessed its significance in intraductal papillary mucinous neoplasm (IPMN) of the pancreas. METHODS: We examined 39 surgically resected specimens of IPMN that included 17 adenomas (IPMAs), 5 borderline tumors (IPMBs), 4 non-invasive carcinomas (non-invasive IPMCs), and 13 invasive carcinomas (invasive IPMCs). Immunostaining was performed according to the EnVision ChemMate method. The degree of maspin expression was scored and assessed according to the percentage and staining intensity of positive cells. RESULTS: Maspin expression was minimal in normal pancreatic duct epithelium, whereas in IPMNs, maspin was expressed in neoplasms of all stages. Maspin expression increased with increasing grade from IPMAs, IPMBs, to non-invasive IPMCs but decreased significantly in invasive IPMCs. No specific association between maspin expression and mucin type was found. Analysis of maspin expression with respect to clinicopathologic factors in cases of invasive IPMC revealed a greater extent of invasion in cases of low maspin expression and significantly fewer apoptotic cells in the tumor.CONCLUSIONS: Maspin was expressed at high levels in IPMNs at various stages from adenoma to invasive carcinoma, and our results suggest that maspin may be involved in the occurrence and progression of IPMN. In addition, our data suggest that the apoptosis-inducing action of maspin suppresses invasion and progression of IPMN.

molecular pathology of intraductal papillary mucinous neoplasms of the pancreas

Since the first description of intraductal papillary mucinous neoplasms(IPMNs)of the pancreas in the eighties,their identification has dramatically increased in the last decades,hand to hand with the improvements in diagnostic imaging and sampling techniques for the study of pancreatic diseases.However,the heterogeneity of IPMNs and their malignant potential make difficult the management of these lesions.The objective of this review is to identify the molecular characteristics of IPMNs in order to recognize potential markers for the discrimination of more aggressive IPMNs requiring surgical resection from benign IPMNs that could be observed.We briefly summarize recent research findings on the genetics and epigenetics of intraductal papillary mucinous neoplasms,identifying some genes,molecular mechanisms and cellular signaling pathways correlated to the pathogenesis of IPMNs and their progression to malignancy.The knowledge of molecular biology of IPMNs has impressively developed over the last few years.A great amount of genes functioning as oncogenes or tumor suppressor genes have been identified,in pancreatic juice or in blood or in the samples from the pancreatic resections,but further researches are required to use these informations for clinical intent,in order to better define the natural history of these diseases and to improve their management.

Simultaneous intraductal papillary neoplasms of the bile duct and pancreas treated with chemoradiotherapy

Some authors have suggested that intraductal papillary mucinous neoplasms of the bile duct(IPMN-B) could be the the biliary counterpart of IPMN of the pancreas(IPMN-P) since they share several clinical-pathological features.These include prominent intraductal papil-lary proliferation pattern,a gastrointestinal phenotype,frequent mucin hyper-secretion and progression to mu-cinous carcinoma.To date there are just four reported cases of patients with synchronous IPMN-B and IPMN-P all of which were treated surgically.We hereby report the case of a 76-year-old woman who was incidentally diagnosed with both an asymptomatic 3 cm bulky uid lesion obstructing the bile duct lumen,diagnosed as a malignant IPMN-B,and synchronous multiple pancreatic cystic lesions(10-13 mm) communicating with an irreg-ular Wirsung,diagnosed as branch duct IPMN-P.Since surgery was ruled-out because of the patient's age and preferences,she underwent a conservative manage-ment regimen comprising both chemotherapy and radio-therapy.This was effective in decreasing the mass size and in resolving subsequent jaundice.This is also the f irst reported case of IPMN-B successfully treated with chemoradiotherapy.Clinicians should consider medical treatment as an option in this clinical scenario,in pa-tients who may be unf it for surgery.

Duodenum and ventral pancreas preserving subtotal pancreatectomy for low-grade malignant neoplasms of the pancreas: An alternative procedure to total pancreatectomy for low-grade pancreatic neoplasms

AIM To describe the indications, technique and outcomes of the novel surgical procedure of duodenum and ventral pancreas preserving subtotal pancreatectomy(DVPPSP).METHODS Data collected retrospectively from 43 patients who underwent DVPPSP and TP between 2009 and 2015 in our single centre were analysed. For enrolment, only patients with low-grade pancreatic neoplasms, such as pancreatic neuroendocrine tumors, intraductal papillary mucinous neoplasms(IPMNs), and solid pseudopapillary tumors, were included. Ten DVPPSP(group 1) and 13 TP(group 2) patients were selected in this study.RESULTS There were no significant differences in age, gender, comorbidities, preoperative symptoms, American Society of Anesthesiologists score or indications for surgery between the two groups. The most common indication was IPMN for DVPPSP and TP(60% vs 85%, P = 0.411). Compared with the TP group, the DVPPSP group had comparable postoperative morbidities(P = 0.405) and mortalities(both nil), but significantly shorter operative time(232 ± 19.6 min vs 335 ± 32.3 min, P < 0.001). DVPPSP preserved better long-term pancreatic function with less supplementary therapy(P < 0.001) and better quality of life(Qo L) after surgery, including better scores in social(P = 0.042) and global health(P = 0.047) on functional scales and less appetite loss(P = 0.049) on the symptom scale. CONCLUSION DVPPSP is a feasible and safe procedure that could be an alternative to TP for low-grade neoplasms arising from the body and tail region but across the neck region of the pancreas; DVPPSP had better metabolic function and Qo L after surgery.

Management of mucinous cystic neoplasms of the pancreas

The purpose of this study was to investigate the actual management of mucinous cystic neoplasm (MCN) of the pancreas. A systematic review was performed in December 2009 by consulting PubMed MEDLINE for publications and matching the "pancreatic mucinous cystic neoplasm", "pancreatic mucinous cystic tumour", "pancreatic mucinous cystic mass", "pancreatic cyst", and "pancreatic cystic neoplasm" to identify English language articles describing the diagnosis and treatment of the mucinous cystic neoplasm of the pancreas. In total, 16 322 references ranging from January 1969 to December 2009 were analysed and 77 articles were identified. No articles published before 1996 were selected because MCNs were not previously considered to be a completely autonomous disease. Definition, epidemiology, anatomopathological findings, clinical presentation, preoperative evaluation, treatment and prognosis were reviewed. MCNs are pancreatic mucinproducing cysts with a distinctive ovarian-type stroma localized in the body-tail of the gland and occurring in middle-aged females. The majority of MCNs are slow growing and asymptomatic. The prevalence of invasive carcinoma varies between 6% and 55%. Preoperative diagnosis depends on a combination of clinical features, tumor markers, computed tomography (CT), magnetic resonance imaging, endoscopic ultrasound with cyst fluid analysis, and positron emission tomography-CT. Surgery is indicated for all MCNs.

Nomograms predicting long-term survival in patients with invasive intraductal papillary mucinous neoplasms of the pancreas: A population-based study

BACKGROUND There are few effective tools to predict survival in patients with invasive intraductal papillary mucinous neoplasms of the pancreas.AIM To develop comprehensive nomograms to individually estimate the survival outcome of patients with invasive intraductal papillary mucinous neoplasms of the pancreas.METHODS Data of 1219 patients with invasive intraductal papillary mucinous neoplasms after resection were extracted from the Surveillance,Epidemiology,and End Results database,and randomly divided into the training(n=853)and the validation(n=366)cohorts.Based on the Cox regression model,nomograms were constructed to predict overall survival and cancer-specific survival for an individual patient.The performance of the nomograms was measured according to discrimination,calibration,and clinical utility.Moreover,we compared the predictive accuracy of the nomograms with that of the traditional staging system.RESULTS In the training cohort,age,marital status,histological type,T stage,N stage,M stage,and chemotherapy were selected to construct nomograms.Compared with the American Joint Committee on Cancer 7th staging system,the nomograms were generally more discriminative.The nomograms passed the calibration steps by showing high consistency between actual probability and nomogram prediction.Categorial net classification improvements and integrated discrimination improvements suggested that the predictive accuracy of the nomograms exceeded that of the American Joint Committee on Cancer staging system.With respect to decision curve analyses,the nomograms exhibited more preferable net benefit gains than the staging system across a wide range of threshold probabilities.CONCLUSION The nomograms show improved predictive accuracy,discrimination capability,and clinical utility,which can be used as reliable tools for risk classification and treatment recommendations.

Cystic neoplasms of the pancreas: A diagnostic challenge

Cystic neoplasms of the pancreas are increasingly recognized due to the expanding use and improved sensitivity of cross-sectional abdominal imaging. Major advances in the last decade have led to an improved understanding of the various types of cystic lesions and their biologic behavior. Despite signifi cant improvements in imaging technology and the advent of endoscopic-ultrasound (EUS)-guided fineneedle aspiration, the diagnosis and management of pancreatic cystic lesions remains a significant clinical challenge. The fi rst diagnostic step is to differentiate between pancreatic pseudocyst and cystic neoplasm. If a pseudocyst has been effectively excluded, the cornerstone issue is then to determine the malignant potential of the pancreatic cystic neoplasm. In the majority of cases, the correct diagnosis and successful management is based not on a single test but on incorporating data from various sources including patient history, radiologic studies, endoscopic evaluation, and cyst fluid analysis. This review will focus on describing the various types of cystic neoplasms of the pancreas, their malignant potential, and will provide the clinician with a comprehensive diagnostic approach.

Extrapancreatic malignancies and intraductal papillary mucinous neoplasms of the pancreas

Over the last two decades multiple studies have demonstrated an increased incidence of additional malignancies in patients with intraductal papillary mucinous neoplasms(IPMNs).Additional malignancies have been identified in 10%-52% of patients with IPMNs.The majority of these additional cancers occur before or concurrent with the diagnosis of IPMN.The gastrointestinal tract is most commonly involved in secondary malignancies,with benign colon polyps and colon cancer commonly seen in western countries and gastric cancer commonly seen in Asian countries.Other extrapancreatic malignancies associated with IPMNs include benign and malignant esophageal neoplasms,gastrointestinal stromal tumors,carcinoid tumors,hepatobiliary cancers,breast cancers,prostate cancers,and lung cancers.There is no clear etiology for the development of secondary malignancies in patients with IPMN.Although population-based studies have shown different results from single institution studies regarding the exact incidence of additional primary cancers in IPMN patients,both have reached the same conclusion:there is a higher incidence of extrapancreatic malignancies in patients with IPMNs than in the general population.This f inding has signif icant clinical implications for both the initial evaluation and the subsequent long-term followup of patients with IPMNs.If a patient has not had recent colonoscopy,this should be performed during the evaluation of a newly diagnosed IPMN.Upper endoscopy should be performed in patients from Asian countries or for those who present with symptoms suggestive of upper gastrointestinal disease.Routine screening studies(breast and prostate) should be carried out as currently recommended for patient's age both before and after the diagnosis of IPMN.

Prospects and applications of enucleation in solid pseudopapillary neoplasms of the pancreas

Solid pseudopapillary neoplasms(SPNs)of the pancreas are rare,low-grade,malignant neoplasms that are mostly seen in young women in the second and third decades of life and are quite uncommon in children.Standard resection for benign and borderline neoplasms of the pancreas is associated with a substantial risk of postoperative morbidity and long-term functional impairment,whereas enucleation leads to less morbidity and preserves healthy parenchyma as well as exocrine and endocrine function.Enucleation of SPNs has been increasingly reported to be feasible and safe for preserving the normal physiological function of the pancreas,especially in teenagers and children.This review summarizes findings published in recent years on the enucleation of SPNs as well as potential future developments and directions.Enucleation has undoubtedly come to stay as an alternative surgical procedure for SPNs.However,many questions remain unresolved,and future directions toward the best surgical indication,the prevention and intervention of complications,especially pancreatic fistula,intraoperative resection margin safety assessment,and long-term oncology prognosis remain to be evaluated and should be explored in future clinical trials.

Yield of surgery in solid pseudopapillary neoplasms of the pancreas: A case series and literature review

BACKGROUND Solid pseudopapillary neoplasms(SPN)of the pancreas represents approximately 2%of non-endocrine tumors of the pancreas.It is described in the literature as a rare and predominant tumor in young women.AIM To report a case series with SPN and analyzing clinical,surgical,anatomopathological characteristics,as well as the prognosis and review of literature.METHODS Retrospective analysis of patients undergoing surgery,with histological diagnosis of SPN between 1998 and 2018,using standardized and prospectively completed forms,performed at the Surgery Service of the Upper Digestive System at Hospital São Rafael/Rede D’Or in Salvador-BA.Review of literature through a database search in MEDLINE/PubMed of retrospective articles.RESULTS Fourteen female patients with the average age of 31.6 years(range min-max)were selected.Twelve patients(85.7%)were asymptomatic,being an incidental diagnosis or due to screening for other reasons.One patient had abdominal pain due to gastric compression and another patient had jaundice.The 14 patients were staged with computerized tomography or magnetic resonance imaging.None had evidence of metastasis.In 8 patients(57.1%),the tumor was in the tail and body.The average size was 6.7 cm(range min-18).The type of surgery was according to the anatomical location of the tumor.There was no lymph node involvement.In two cases,vascular resection with the use of a prosthesis was required for reconstruction.The surgical margins were free.In all cases,postoperative immunohistochemistry confirmed that it was a solid pseudo-papillary neoplasia of the pancreas.There has been no disease recurrence in any case so far.CONCLUSION The tumors had a benign,indolent and histopathological behavior compatible with the literature.Curative surgery is recommended in all cases.