Next-generation sequencing in the evaluation of biliary strictures in patients with primary sclerosing cholangitis.

BACKGROUND: Primary sclerosing cholangitis (PSC) is a well-described risk factor for the development of cholangiocarcinoma (CCA). Early detection of CCA in these patients is of great importance because it expands options for therapeutic interventions, including liver transplantation. Current diagnostic tests for the evaluation of biliary strictures are limited to biliary brushing (BB) cytology and fluorescence in situ hybridization (FISH). Next-generation sequencing (NGS) has become an important diagnostic tool in oncology and may be a useful tool for diagnosing CCA on BBs. It is not clear how NGS performs when it is added to BB cytology and FISH in patients with PSC. METHODS: This study reports the authors' experience with NGS performed as a prospective cotest with cytology and FISH on BBs obtained from 60 patients with PSC followed at Massachusetts General Hospital. A duct with malignancy was defined as a high-risk (HR) stricture with either high-grade dysplasia or CCA. RESULTS: NGS was better than FISH and cytology in detecting HR strictures, which showed multiple genetic mutations in all cases. NGS provided specific mutational information, and NGS results were reproducible in longitudinal samples. CONCLUSIONS: Adding NGS to BB cytology and FISH in the evaluation of biliary strictures for patients with PSC may provide additional information that could help to inform clinical management.

Endoscopic ultrasound-guided fiducial marker placement in pancreatic cancer: A systematic review and meta-analysis.

BACKGROUND: Pancreatic cancer (PC) mortality remains high despite advances in therapy. Combination chemoradiotherapy offers modest survival benefit over monotherapy with either. Fiducial markers serve as needed landmarks for image-guided radiotherapy (IGRT). Traditionally, these markers were placed surgically or percutaneously with limitations of each. Endoscopic ultrasound-guided placement overcomes these limitations. AIM: To evaluate the safety, efficacy, and feasibility of endoscopic ultrasound (EUS)-guided fiducial placement for PC undergoing IGRT. METHODS: Articles were searched in MEDLINE, PubMed, and Ovid journals. Pooling was conducted by fixed and random effects models. Heterogeneity was assessed using Cochran's Q test based upon inverse variance weights. RESULTS: Initial search identified 1024 reference articles for EUS-guided fiducial placement in PC. Of these, 261 relevant articles were reviewed. Data was extracted from 11 studies (n = 820) meeting inclusion criteria. Pooled proportion of successful placement was 96.27% (95%CI: 95.35-97.81) with fiducial migration rates low at 4.33% (95%CI: 2.45-6.71). Adverse event rates remained low, with overall pooled proportion of 4.85% (95%CI: 3.04-7.03). CONCLUSION: EUS-guided placement of fiducial markers for IGRT of PC is safe, feasible, and efficacious. The ability to target deep structures under direct visualization while remaining minimally invasive are added benefits. Moreover, the ability to perform fine needle aspiration or celiac plexus neurolysis add value and increase patient-care efficiency. Whether EUS-guided fiducial placement improves outcomes in IGRT or offers any mortality benefits over traditional placement remains unknown and future studies are needed.

Improved Mortality in Necrotizing Pancreatitis with a Multidisciplinary Minimally Invasive Step-Up Approach: Comparison with a Modern Open Necrosectomy Cohort.

BACKGROUND: A minimally invasive step-up (MIS) approach has been associated with reduced morbidity compared with open surgical necrosectomy (OSN) for treatment of necrotizing pancreatitis. We sought to determine whether transitioning from an OSN to an MIS-based approach would result in reduced mortality. MIS interventions included percutaneous drainage, endoscopic transgastric necrosectomy, video-assisted retroperitoneal debridement, sinus tract endoscopic necrosectomy, or a combination of techniques, with selective use of OSN. STUDY DESIGN: We conducted an observational cohort study with retrospective comparison at a single tertiary referral center (2006 through 2019). Eighty-eight patients were treated with OSN and 91 were treated with an MIS-based approach. Baseline characteristics and clinical outcomes were compared between groups. The primary end point was 90-day mortality. RESULTS: There was no difference in baseline characteristics. Ninety-day mortality was 2% with MIS compared with 10% with OSN (p = 0.03). One-year mortality was 3% with MIS compared with 15% with OSN (p = 0.012). The rate of organ failure was lower with MIS (30% vs 45%; p = 0.029), but there was a higher bleeding rate (19% vs 9%; p = 0.064). In the MIS group, 9% were treated with percutaneous drainage, 32% with endoscopic transgastric necrosectomy, 8% with video-assisted retroperitoneal debridement, 15% with sinus tract endoscopic necrosectomy, and 27% with a combination of techniques. CONCLUSIONS: Adoption of a multidisciplinary MIS-based approach to necrotizing pancreatitis resulted in a 5-fold decrease in mortality compared with OSN.

Peroral cholangioscopy with cholangioscopy-directed biopsies in the diagnosis of biliary malignancies: a systemic review and meta-analysis.

BACKGROUND AND AIM: Accurate diagnosis is essential in the appropriate management of biliary strictures. Our aim is to evaluate the efficacy of cholangioscopy-directed biopsies in differentiating biliary intraductal malignancies from benign lesions. MATERIALS AND METHODS: Articles were searched in Medline, PubMed, and Ovid journals. Pooling was performed by both fixed-effects and random-effects models. Only studies from which a 2×2 table could be constructed for true-positive, false-negative, false-positive, and true-negative values were included. RESULTS: Initial search identified 2110 reference articles for peroral cholangioscopy; of these, 160 relevant articles were selected and reviewed. Data were extracted from 15 studies (N=539) that fulfilled the inclusion criteria. Pooled sensitivity of cholangioscopy-directed biopsies in diagnosing malignancy was 71.9% [95% confidence interval (CI): 66.1-77.1] and pooled specificity was 99.1% (95% CI: 96.9-99.9). The positive likelihood ratio of cholangioscopy-directed biopsies was 18.1 (95% CI: 9.1-35.8), whereas the negative likelihood ratio was 0.3 (95% CI: 0.2-0.4). The pooled diagnostic odds ratio was 71.6 (95% CI: 32.8-156.4). All the pooled estimates calculated by fixed-effects and random-effect models were similar. Summary receiver operating characteristic curves showed an area under the curve of 0.98. The χ heterogeneity for all the pooled accuracy estimates was 5.62 (P=0.96). CONCLUSION: Peroral cholangioscopy with cholangioscopy-directed biopsies has a high specificity in differentiating intraductal malignancies from benign lesions. Cholangioscopy-directed biopsies should be strongly considered for biliary stricture evaluation.

Preoperative endoscopic retrograde cholangio-pancreatography (ERCP) is a risk factor for surgical site infections after laparoscopic cholecystectomy.

BACKGROUND: We sought to examine whether preoperative endoscopic retrograde cholangio-pancreatography (ERCP) increases the risk of surgical site infections (SSI) after laparoscopic cholecystectomy. METHODS: Patients admitted to an academic hospital from 2010 to 2016, who were older than 18 and had a laparoscopic or a laparoscopic converted to open cholecystectomy for complicated biliary tract disease were included. We compared those who had a preoperative ERCP to those who did not. Our primary endpoint was the rate of SSI. RESULTS: A total of 640 patients were included. Of them, 122 (19.1%) received preoperative ERCP and 518 (80.9%) did not. The former had different preoperative diagnoses compared to non-ERCP patients (choledocholithiasis [35.2%-7.0%], acute cholecystitis [31.2%-76.4%], gallstone pancreatitis [20.5%-16.2%], and cholangitis [13.1%-0.4%], p < 0.001). The rate of SSI was higher in the preoperative ERCP group (11.5%-4.0%, p = 0.005). In a multivariable analysis conversion to open (OR = 2.57, 95% CI = 1.06-6.21, p = 0.037) and preoperative ERCP (OR = 3.12, 95% CI = 1.34-7.22, p = 0.008) were the only independent predictors of SSI. CONCLUSION: Preoperative ERCP is associated with a threefold increase in the risk of SSI after laparoscopic cholecystectomy.

Moray micro forceps biopsy improves the diagnosis of specific pancreatic cysts.

BACKGROUND: Making a specific diagnosis of pancreatic cysts preoperatively is difficult. The new disposable Moray micro forceps biopsy (MFB) device allows tissue sampling from the pancreatic cyst wall/septum and aims to improve diagnosis. This study compares the diagnostic performance of the MFB with the current conventional analysis of pancreatic cyst fluid (PCF). METHODS: A total of 48 patients sampled with MFB were identified. Cysts were classified as mucinous on PCF based on extracellular mucin/mucinous epithelium, carcinoembryonic antigen (CEA) levels ≥192 ng/mL, or KRAS/GNAS mutation. A diagnosis of intraductal papillary mucinous neoplasm was supported by GNAS mutation; a diagnosis of serous cystadenoma was supported by Von Hippel-Lindau tumor suppressor (VHL) mutation. A diagnosis of mucinous cystic neoplasm required the presence of subepithelial ovarian-type stroma. A high-risk cyst was defined as a mucinous cyst with high-grade dysplasia or an adenocarcinoma. Comparisons in diagnostic performance between PCF and MFB were made. RESULTS: The mean age of the patients was 69.6 years (range, 27-90 years); 25 of 48 patients (52.1%) were female. Cysts were in the pancreatic head (13 patients), neck (2 patients), body (20 patients), and tail (13 patients), averaging 3.1 cm (range, 1.2-6.0 cm). There was concordance with mucinous versus nonmucinous classification (60.4% for PCF vs 58.3% for MFB; P = .949). Three high-risk cysts were detected by PCF and 2 were detected by MFB (P = .670). However, MFB diagnosed significantly more specific cysts compared with PCF (50.0% for MFB vs 18.8% for PCF; P<.001). CONCLUSIONS: PCF analysis and MFB have comparable performance in distinguishing between mucinous and nonmucinous cysts and for detecting high-risk cysts. However, MFB was found to be superior for diagnosing specific cyst subtypes, thus adding significant value to preoperative patient management. Cancer Cytopathol 2018;126:414-20. © 2018 American Cancer Society.

Needle-Based Confocal Laser Endomicroscopy for Evaluation of Cystic Neoplasms of the Pancreas.

BACKGROUND: The accurate diagnosis of cystic neoplasms of the pancreas (CNP) with current diagnostic methods is limited. Endoscopic ultrasound (EUS)-guided needle-based confocal laser endomicroscopy (nCLE) is a new technique which can obtain images from the cyst wall during EUS-fine needle aspiration (EUS-FNA). The aim of this study was to assess the safety, feasibility, and diagnostic value of nCLE for CNP. METHODS: Patients who underwent EUS-FNA to evaluate a CNP larger than 2 cm were enrolled. The cyst was punctured with 19-G FNA needle preloaded with an nCLE probe. The images from different areas of the cyst wall were recorded. Using the final diagnosis defined by surgery or EUS-FNA cyst fluid analysis, the accuracy of the confocal images was defined. RESULTS: The procedure and image acquisition was successful in 18 of the 20 patients. Predefined typical structures for mucinous cysts were visualized in 8 of 12 (66%) cysts but none of the non-mucinous cysts. The superficial vascular network which is a typical finding of serous cysts was observed in 2 of 3 patients. The sensitivity, specificity, and diagnostic accuracy of the findings of epithelial structures by nCLE were 66, 100, and 80%, respectively, for a mucinous cyst diagnosis. All patients tolerated the procedure well, and no adverse effects were determined. CONCLUSION: nCLE was found to be safe and feasible with high technical success, in this pilot study. With an overall accuracy of 80%, it has the potential to contribute to the diagnosis of CNP with specific imaging.

Value of adding GNAS testing to pancreatic cyst fluid KRAS and carcinoembryonic antigen analysis for the diagnosis of intraductal papillary mucinous neoplasms.

BACKGROUND AND AIM: Molecular analysis of pancreatic cyst fluid (PCF) has been proposed as a novel method for differentiating pancreatic cystic lesions (PCL). The present study aimed to investigate the value of GNAS testing when added to KRAS and carcinoembryonic antigen (CEA) testing of PCF for the diagnosis of intraductal papillary mucinous neoplasms (IPMN). METHODS: Prospectively collected endoscopic ultrasonography fine-needle aspiration (EUS-FNA) data were analyzed retrospectively for GNAS and KRAS mutations and CEA results. IPMN were histologically confirmed or supported by imaging and EUS-FNA findings (KRAS, CEA, cytology). Performance characteristics of GNAS added to KRAS and CEA for the diagnosis of IPMN were calculated. RESULTS: The study population consisted of 197 patients with cyst fluid test results. Cysts were histologically classified in 33 patients and by clinical criteria in 164 patients. The IPMN group included 108 patients and the non-IPMN group included 89 patients. GNAS was positive in 51 patients (47.2%) with IPMN. Forty-two of these patients (82.3%) also had a KRAS mutation. Adding GNAS to KRAS increased the diagnostic accuracy from 76.6% to 79.1% (P > 0.05). Adding GNAS to CEA increased the diagnostic accuracy from 66.4% to 80.7 % (P < 0.05), but did not achieve a diagnostic superiority to KRAS testing alone (80.7% vs 76.6%, P > 0.05). The diagnostic accuracy of the triple combination was significantly better than all single tests (P < 0.05). CONCLUSION: GNAS mutation is a highly specific test for IPMN. When GNAS testing is added to CEA and KRAS, a significantly greater overall accuracy (86.2%) is achieved.

Radiofrequency ablation for the management of occluded biliary metal stents.

Background and study aim: Obstruction of biliary self-expandable metal stents (SEMSs) is seen frequently. Radiofrequency ablation (RFA) causes tissue necrosis. This study aimed to assess the efficacy of RFA for management of occluded SEMS. Patients and methods: Patients with biliary malignancy and treated for an occluded SEMS were retrospectively reviewed. The study group comprised patients treated with RFA using an Habib endoprobe inside the SEMS. The control group comprised patients treated only with insertion of a plastic stent into an occluded SEMS. The end points were; 90-day stent patency rate, time to stent reocclusion, 30-day mortality, and 3 - and 6-month survival rates. Results: During the 5-year study period 25 patients with an occluded SEMS underwent RFA and 25 patients underwent a plastic stent placement only. Both groups were matched for age and diagnosis. Immediate biliary drainage was restored in all patients. In the RFA group, the stenosis was ablated successfully in 14 patients (56 %); ablation failed in 11 and a plastic stent was then inserted into the SEMS for these. The control group had only placement of a plastic stent across the stenosis. Stent patency rate at 90 days was 56 % and 24 % in the RFA and control groups, respectively (P = 0.04). The mean stent patency time was significantly longer in the RFA group compared to the control group (119.5 vs. 65.3 days, P = 0.03). Conclusion: The application of RFA for occluded SEMS improves stent patency. RFA is an alternative treatment of tissue ingrowth in malignant biliary obstruction.

Combined endoscopic trangastric drainage and video assisted retroperitoneal pancreatic debridement - The best of both worlds for extensive pancreatic necrosis with enteric fistulae.

Enteric fistula is a serious complication of necrotizing pancreatitis. Endoscopic transluminal drainage and necrosectomy can significantly reduce the incidence of enterocutaneous fistula after pancreatic debridement. However, endoscopic necrosectomy may not be well-suited to debridement of necrosis that tracks laterally to the paracolic gutters, which is often more efficiently addressed by video-assisted retroperitoneal debridement (VARD). We report the combined use of endoscopic transgastric drainage and VARD for treatment of a 76 year old man with severe necrotizing acute pancreatitis complicated by infected, walled-off pancreatic necrosis. Computed tomography showed laterally tracking pancreatic necrosis and flouroscopic drain injection after percutaneous drainage demonstrated with fistulas to the stomach, duodenum, and colon. The infection and fistulas resolved completely. This approach combined the major advantage of VARD with the major advantage of endoscopic transluminal drainage. We are not aware of any reports of combining these techniques and believe the combination offers a minimally invasive approach for patients with extensive necrosis and a high likelihood of enteric or pancreatic fistulas.

Endoscopic Therapy With Lumen-apposing Metal Stents Is Safe and Effective for Patients With Pancreatic Walled-off Necrosis.

BACKGROUND & AIMS: Endoscopic ultrasound-guided transmural drainage and necrosectomy have become the standard treatment for patients with pancreatic walled-off necrosis (WON). Lumen-apposing metal stents (LAMS) have shown success in the management of pancreatic fluid collections. However, there are few data on their specific roles in management of WON. We investigated the efficacy and safety of LAMS in treatment of WON. METHODS: We performed a retrospective multicenter case series of 124 patients with WON who underwent endoscopic transmural drainage by using LAMS at 17 tertiary care centers from January 2014 through May 2015. Patients underwent endoscopic ultrasound-guided cystogastrostomy or cystoenterostomy with placement of an LAMS into the WON collection. At the discretion of the endoscopist, we performed direct endoscopic necrosectomy, irrigation with hydrogen peroxide, and/or nasocystic drain placement. We performed endoscopic retrograde cholangiopancreatography with pancreatic duct stent placement when indicated. Concomitant therapies included direct endoscopic debridement (n = 78), pancreatic duct stent placement for leak (n = 19), hydrogen peroxide-assisted necrosectomy (n = 38), and nasocystic irrigation (n = 22). We collected data for a median time of 4 months (range, 1-34 months) after the LAMS placement. The primary outcomes were rates of technical success (successful placement of the LAMS), clinical success (resolution of WON, on the basis of image analysis, without need for further intervention via surgery or interventional radiology), and adverse events. RESULTS: The median size of the WON was 9.5 cm (range, 4-30 cm). Eight patients had 2 LAMS placed for multiport access, all with technical success (100%). Clinical success was achieved in 107 patients (86.3%) after 3 months of follow-up. Thirteen patients required a percutaneous drain, and 3 required a surgical intervention to manage their WON. The stents remained patent in 94% of patients (117 of 124) and migrated in 5.6% of patients (7 of 124). The median number of endoscopic interventions was 2 (range, 1-9 interventions). CONCLUSIONS: On the basis of a retrospective analysis of 124 patients, endoscopic therapy of WON by using LAMS is safe and effective. Creation of a large and sustained cystogastrostomy or cystoenterostomy tract is effective in the drainage and treatment of WON.

The value of KRAS mutation testing with CEA for the diagnosis of pancreatic mucinous cysts.

BACKGROUND AND AIMS: Pancreatic cyst fluid (PCF) CEA has been shown to be the most accurate preoperative test for detection of cystic mucinous neoplasms (CMNs). This study aimed to assess the added value of PCF KRAS mutational analysis to CEA for diagnosis of CMNs. PATIENTS AND METHODS: This is a retrospective study of prospectively collected endoscopic ultrasonography (EUS) fine-needle aspiration (FNA) data. KRAS mutation was determined by direct sequencing or equivalent methods. Cysts were classified histologically (surgical cohort) or by clinical (EUS or FNA) findings (clinical cohort). Performance characteristics of KRAS, CEA and their combination for detection of a cystic mucinous neoplasm (CMN) and malignancy were calculated. RESULTS: The study cohort consisted of 943 patients: 147 in the surgical cohort and 796 in the clinical cohort. Overall, KRAS and CEA each had high specificity (100 % and 93.2 %), but low sensitivity (48.3 % and 56.3 %) for the diagnosis of a CMN. The positivity of KRAS or CEA increased the diagnostic accuracy (80.8 %) and AUC (0.84) significantly compared to KRAS (65.3 % and 0.74) or CEA (65.8 % and 0.74) alone, but only in the clinical cohort (P < 0.0001 for both). KRAS mutation was significantly more frequent in malignant CMNs compared to histologically confirmed non-malignant CMNs (73 % vs. 37 %, P = 0.001). The negative predictive value of KRAS mutation was 77.6 % in differentiating non-malignant cysts. CONCLUSIONS: The detection of a KRAS mutation in PCF is a highly specific test for mucinous cysts. It outperforms CEA for sensitivity in mucinous cyst diagnosis, but the data does not support its routine use.

Next-Generation Sequencing and Fluorescence in Situ Hybridization Have Comparable Performance Characteristics in the Analysis of Pancreaticobiliary Brushings for Malignancy.

Cytological evaluation of pancreatic or biliary duct brushings is a specific, but insensitive, test for malignancy. We compared adjunctive molecular testing with next-generation sequencing (NGS) relative to fluorescence in situ hybridization (FISH) for detection of high-risk neoplasia or malignancy. Bile duct brushings from 81 specimens were subjected to cytological analysis, FISH using the UroVysion probe set, and targeted NGS. Specimens were placed into negative/atypical (negative) or suspicious/positive (positive) categories depending on cytology and negative or positive categories on the basis of FISH and NGS results. Performance characteristics for each diagnostic modality were calculated on the basis of clinicopathologic follow-up and compared in a receiver operating characteristic analysis. There were 33 high-risk neoplasia/malignant strictures (41%) and 48 benign (59%). NGS revealed driver mutations in 24 cases (30%), including KRAS (21 of 24 cases), TP53 (14 of 24 cases), SMAD4 (6 of 24 cases), and CDKN2A (4 of 24 cases). Cytology had a sensitivity of 67% (95% CI, 48%-82%) and a specificity of 98% (95% CI, 89%-100%). When added to cytology, NGS increased the sensitivity to 85% (95% CI, 68%-95%), leading to a significant increase in the area under the curve in a receiver operating characteristic analysis (P = 0.03). FISH increased the sensitivity to 76% (95% CI, 58%-89%), without significantly increasing the area under the curve. These results suggest that ancillary NGS testing offers advantages over FISH, although studies with larger cohorts are needed to verify these findings.

Predictors Of Treatment Failure After Radiofrequency Ablation For Intramucosal Adenocarcinoma in Barrett Esophagus: A Multi-institutional Retrospective Cohort Study.

Radiofrequency ablation (RFA), with or without endoscopic mucosal resection (EMR), is a safe, effective, and durable treatment option for Barrett esophagus (BE)-associated dysplasia (DYS), but few studies have identified predictors of treatment failure in BE-associated intramucosal adenocarcinoma (IMC). The aim of this study was to determine the rate of IMC eradication when using RFA±EMR and to identify clinical and pathologic predictors of treatment failure. A retrospective review of medical records and a central review of index histologic parameters were performed for 78 patients who underwent RFA±EMR as the primary treatment for biopsy-proven IMC at 4 academic tertiary medical centers. Complete eradication (CE) (absence of IMC/DYS on first follow-up endoscopy) was achieved in 86% of patients, and durable eradication (DE) (CE with no recurrence of IMC/DYS until last follow-up) was achieved in 78% of patients, with significant variation between the 4 study sites (P=0.03 and 0.09 by analysis of variance for DE and CE, respectively). Use of EMR before RFA significantly reduced the risk for treatment failure for IMC/DYS (hazard ratio, 0.15; 95% confidence interval, 0.05-0.48; P=0.001), whereas IMC involving ≥50% of the columnar metaplastic area on index examination significantly increased the risk for treatment failure (hazard ratio, 4.24; 95% confidence interval, 1.53-11.7; P=0.005). Endoscopic and pathologic factors associated with treatment failure in BE-associated IMC treated with RFA±EMR may help identify the subset of IMC patients for whom a more aggressive initial approach may be justified.

Late Pancreatic Fistula After Pancreaticoduodenectomy: A Case Report and Review of the Literature.

Background: More than 100 years after its conception, the pancreaticoduodenectomy (PD) remains a challenging procedure with significant morbidity, often due to a postoperative pancreatic fistula (POPF). Factors related to patient physiology, tumor anatomy/pathology, and surgeon/surgical technique have been studied, yielding results at times conflicting and difficult to reproduce. We present a case of a late POPF along with a brief review of the current literature. Case Presentation: The patient is a 55-year-old female with a 20 pack-year smoking history and no history of alcohol abuse, who presented for evaluation of new nausea. Her laboratory tests and computed tomography (CT) imaging were suggestive of biliary obstruction. She was found to have an invasive ampullary adenocarcinoma and subsequently underwent a classic PD. She developed a POPF, managed through a closed suction drain placed intraoperatively. Her course was complicated by the development of an intra-abdominal abscess, managed percutaneously through CT-guided placement of two drains, subsequently removed without issues. She recovered uneventfully until 8 months after the operation, when she presented with abdominal pain and pancreatitis. She was found to have an intra-abdominal collection, again managed percutaneously via CT-guided drainage. This time, the amylase and lipase levels of the drainage fluid were 21,860 and 86,650 U/L, respectively, and cultures were sterile. Upon workup of her pancreatic fistula, a severe stricture at the pancreaticojejunostomy (PJ) was identified. She underwent endoscopic placement of a Hobbs stent by the GI service. Conclusion: Although commonly diagnosed in the days to weeks after a PD, we present a case of a POPF that manifested 8 months after the initial operation in association with a PJ stricture. This case highlights the importance of considering the diagnosis even months after the operation in a patient who presents with symptoms of pancreatitis and/or imaging findings consistent with an intra-abdominal collection.

Lymphoepithelial cysts and cystic lymphangiomas: Under-recognized benign cystic lesions of the pancreas.

AIM: To identify their diagnostic and prognostic clinical characteristics in a large series. METHODS: Retrospective review of clinicopathologic and imaging characteristics of patients diagnosed with lymphoepithelial cysts and cystic lymphangiomas of the pancreas at Massachusetts General Hospital. RESULTS: Twelve patients were identified between 1/1/1997 and 8/1/2007. Their median age was 55.5 years (range 19-78 years), and 6 were females. The lesion was incidentally discovered in half of the patients. Contrast enhanced computed tomography demonstrated that the cysts had thin walls, without calcifications, pancreatic duct dilation or pancreatic parenchyma invasion. Endoscopic ultrasound with fine needle aspiration (EUS/FNA) confirmed the diagnosis of a lymphoepithelial cyst in 3 patients, one of whom was spared an operation and continues to do well after 6 years. Eleven patients had a resection: 3 pancreaticoduodenectomies, 7 distal pancreatectomies, and 1 enucleation. The median size of the cysts was 3 cm (range 2-20 cm). At a median follow-up of 57 mo no recurrences or other pancreas-related conditions occurred. CONCLUSION: Lymphoepithelial cysts and cystic lymphangiomas of the pancreas can be diagnosed with a combination of contrast-enhanced computed tomography scans and EUS/FNA. If the lesion is asymptomatic, an operation might be avoided.