Pancreatic panniculitis secondary to pancreatic cyst with lower extremity wounds as a primary presenting feature: a case report and literature review.

INTRODUCTION: Pancreatic panniculitis is a rare skin manifestation of pancreatic disease. It is characterized by inflammation and liquefactive necrosis of subcutaneous fat. Treatment involves addressing the underlying cause and providing supportive wound care. CASE REPORT: The authors present a case of a 68-year-old man who developed painful, erythematous wounds on his lower extremities that progressed to purple, edematous lesions with purulent drainage. During the progression of his wounds, he developed epigastric pain and acute pancreatitis. Subsequent CT scan showed a pancreatic cyst that had extended into the portal vein. Deep, excisional biopsy of the wounds helped further narrow the differential. Histology indicated "ghost cells," which are adipocytes with a central clearing and dark basophilic calcium deposits in the cytoplasm. CONCLUSION: The presence of ghost adipocytes is a rather unique histopathological feature consistent with pancreatic panniculitis and should be considered in combination with the overall clinical picture to determine the underlying etiology. Pancreatic panniculitis can be a primary presenting feature and possible complication of pancreatic disease.

Pancreatic Cystic Lesions on MRI: What Is The Likelihood of a Present or Future Diagnosis of Pancreatic Carcinoma?

BACKGROUND: Pancreatic cystic lesions (PCLs) are followed for years due to older and likely biased works demonstrating a strong association with pancreatic carcinoma; more recent data are needed clarifying this relationship. PURPOSE: To determine the association between PCLs on MRI and a synchronous or future diagnosis of pancreatic carcinoma. STUDY TYPE: Single-center retrospective cohort. POPULATION: A total of 192 patients (111 female, 58%) with median age 66 years (range 26-87 years) with PCLs on abdominal MRI from 2011 to 2016. FIELD STRENGTH/SEQUENCES: 1.5 T and 3 T, including T2 WI, T1 WI, diffusion weighted imaging and contrast-enhanced T1 WI. ASSESSMENT: Each PCL was reviewed independently by 2 of 10 fellowship-trained abdominal radiologists. Fukuoka guideline worrisome features and high-risk stigmata were evaluated. Follow-up imaging and clinical notes were reviewed within a system that captures pancreatic carcinoma for the region, for a median follow-up of 67 months (interquartile range: 43-88 months). STATISTICAL TESTS: Pancreatic carcinoma prevalence and incidence rate for future carcinoma with 95% confidence intervals (95% CI). Fisher exact test, logistic regression with odds ratios (OR) and the Wilcoxon rank-sum test were used to assess PCL morphologic features with the Kolmogorov-Smirnov test used to assess for normality. P < 0.05 defined statistical significance. RESULTS: The prevalence of pancreatic carcinoma on initial MRI showing a PCL was 2.4% (95% CI: 0.9%, 5.2%). Thickened/enhancing cyst wall was associated with pancreatic carcinoma, OR 52 (95% CI: 4.5, 1203). Of 189 patients with a PCL but without pancreatic carcinoma at the time of initial MRI, one developed high-grade dysplasia and none developed invasive carcinoma for an incidence rate of 0.97 (95% CI: 0.02, 5.43) and 0 (95% CI: 0, 3.59) cases per 1000 person-years, respectively. DATA CONCLUSION: A low percentage of patients with a PCL on MRI had a pancreatic carcinoma at the time of initial evaluation and none developed carcinoma over a median 67 months of follow-up. EVIDENCE LEVEL: 3 TECHNICAL EFFICACY: 5.

Diabetes mellitus and the risk of progression or malignancy of pancreatic cystic neoplasms in patients undergoing surveillance: A systematic review and meta-analysis.

BACKGROUND/OBJECTIVES: Patients with pancreatic cystic neoplasms (PCN) generally undergo surveillance by cross-sectional imaging or endoscopic-ultrasound due to their pre-malignant potential. Appearance of certain cyst characteristics during surveillance is associated with aggressive behavior or presence of malignancy. Patient characteristics associated with progression or induction of malignancy in PCN is unclear from current studies. We performed this meta-analysis to determine the patient characteristics associated with progression or malignancy in patients with PCN who undergo surveillance. METHODS: We performed a systematic research of several electronic databases for all the studies on surveillance of PCN which reported demographic data of patients who had progression or malignancy and controls. We calculated Risk ratio (RR) or Hazard ratio (HR) with 95% confidence interval (CI) for each variable. Mantel- Haenszel method or Inverse-variance model was used to pool data of progression or malignant transformation into fixed or random effect model meta-analysis. RESULTS: 11observational studies, 4 assessing risk factors for malignancy (3955 patients) and 7 evaluating risk factors for progression of PCN (3144 patients) were included in the meta-analysis. Diabetes mellitus was associated with higher risk for malignant transformation as well as progression of PCN (RR = 1.54, CI 1.23, 1.92). Advanced age was associated with higher risk of progression. Male gender had higher risk for malignant transformation but not progression of PCN. CONCLUSION: Diabetes is associated with an increased risk of both malignancy and progression of PCN in patients who undergo surveillance. Advanced age is also associated with higher risk of progression of PCN.

Incidence and Risk Factors for New-Onset Diabetes Mellitus After Surgical Resection of Pancreatic Cystic Lesions: A MarketScan Study.

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.

Eosinophilic pancreatitis presenting as rupture of a pancreatic cystic lesion into the chest cavity.

A 71-year-old man was receiving follow-up examination because of a retention cyst in the pancreatic body that extended to the dorsal extrahepatic area, but presented to the Emergency Department at our hospital with dyspnea and cough. Chest X-ray showed a large amount of left-sided pleural effusion and abdominal computed tomography (CT) showed reduction in size of the cystic lesion. Biochemical testing of the pleural effusion revealed high levels of pancreatic enzymes. We, therefore, diagnosed rupture of the pancreatic cystic lesion into the chest cavity. Endoscopic retrograde cholangiopancreatography (ERCP) demonstrated stenosis of the pancreatic duct and leakage of contrast medium at the cystic lesion. CT after ERCP revealed leakage of contrast medium from the cystic lesion through the dorsal extrahepatic area into the chest cavity. Endoscopic naso-pancreatic drainage was performed, but the cystic lesion and pleural effusion remained unimproved. Distal pancreatectomy was, therefore, performed. Microscopic examination revealed eosinophilic infiltration of the pancreatic parenchyma, leading to a diagnosis of eosinophilic pancreatitis (EP). Pancreatic retention cyst secondary to chronic pancreatitis associated with eosinophilic infiltration was considered to have ruptured into the chest cavity. EP is a rare etiology of pancreatitis and few cases have been reported. This case was thus considered valuable.

Multiparameter Analysis Using 18F-FDG PET/CT in the Differential Diagnosis of Pancreatic Cystic Neoplasms.

Purpose: To evaluate multiparametric analysis in differential diagnosis between pancreatic serous cystic neoplasms (SCNs) and mucinous cystic neoplasms (MCNs) as well as the differentiation of the benign and malignant MCNs with 18F-FDG (18-fluorodeoxyglucose) PET/CT (positron emission tomography). Methods: Forty patients with total of 41 lesions (SCNs: 27/41; MCNs: 14/41), who were preoperatively examined with 18F-FDG PET/CT, were retrospectively analyzed. Multiple quantitative parameters using conventional and texture features were included. The combined model was established with complementary PET/MR parameters. The differential diagnostic efficacy of each independent parameter and the combined model were evaluated with receiver operating characteristic (ROC) analysis. Integrated discriminatory improvement (IDI) and net reclassification improvement (NRI) were used to evaluate improvement of diagnostic efficacy by using combination of multiple parameters. Results: Among all independent parameters, the percentile 5th (0.88 ± 0.38 vs 0.47 ± 0.23, P < 0.001) showed the highest discriminative diagnostic value. The combination of multiple parameters can improve the differential diagnostic efficacy of SCNs and MCNs (sensitivity = 71.4%, specificity = 77.8%, and AUC = 0.788), and the addition of texture parameters to the conventional parameters allowed a significant reclassification with IDI = 0.236 (95% CI: 0.095-0.377) and categorical NRI = 0.434 (95% CI: 0.030-0.838). SURmax (tumor to normal pancreas ratio, T/P) and SURmax (tumor to aorta ratio, T/A) both showed the highest discriminative diagnostic value (sensitivity = 100.0%, specificity = 70.0%, AUC = 0.900, and Youden index = 0.700) in the differential diagnosis of benign and malignant MCNs, with the cutoff values of 0.84 and 0.90, respectively. Conclusion: Combination of multiple parameters using 18F-FDG PET/CT could further improve differentiation between pancreatic SCNs and MCNs. SURmax (T/P) and SURmax (T/A) could improve differential diagnosis of benign and malignant MCNs.

New onset diabetes predicts progression of low risk pancreatic mucinous cysts.

BACKGROUND: Patients with low-risk lesions require ongoing surveillance since the rate of progression to pancreatic cancer (PC), while small, is much greater than in the general population. Our objective was to study the relationship between new onset diabetes (NODM) and progression in patients with low risk mucinous cysts. METHODS: We evaluated a prospectively maintained cohort of 442 patients with a suspected mucinous cyst without worrisome features (WF) or high-risk stigmata (HRS). Multivariable Cox models were developed for progression to WF and HRS, with diabetes status formulated as both time independent and dependent covariates. The adjusted cumulative risk of progression was calculated using the corrected group prognosis method. RESULTS: The 5-year cumulative progression rates to WFs and HRS were 12.8 and 3.6%, respectively. After controlling for other risk factors, the development of NODM was strongly associated with progression to HRS (HR = 11.6; 95%CI, 3.5-57.7%), but not WF. Among patients with the smallest cysts (<10 mm) at baseline, those who developed NODM had a 5-year adjusted cumulative risk of progression to HRS of 8.6% (95%CI, 0.0%-20.2%), compared to only 0.8% (95%CI, 0.0%-2.3%) for patients without NODM. Among patients with the largest cysts (20-29 mm), those who developed NODM during surveillance had a 5-year adjusted cumulative risk of progression of 53.5% (95%CI, 19.6%-89.9%) compared to only 7.5% (95%CI, 1.6%-15.2%) for patients without NODM. CONCLUSION: New onset diabetes may predict progression in patients with low risk mucinous cysts. Pending validation with large-scale studies, these findings support regular diabetes screening among patients surveilled for suspected IPMNs or MCNs.

Risk of malignancy in small pancreatic cysts decreases over time.

BACKGROUND: Pancreatic cysts <15 mm without worrisome features have practically no risk of malignancy at the time of diagnosis but this can change over time. Optimal duration of follow-up is a matter of debate. We evaluated predictors of malignancy and attempted to identify a time to safely discontinue surveillance. METHODS: Bi-centric study utilizing prospectively collected databases of patients with pancreatic cysts measuring <15 mm and without worrisome features who underwent surveillance at the Massachusetts General Hospital (1988-2017) and at the University of Verona Hospital Trust (2000-2016). The risk of malignant transformation was assessed using the Kaplan-Meier method and parametric survival models, and predictors of malignancy were evaluated using Cox regression. RESULTS: 806 patients were identified. Median follow-up was 58 months (6-347). Over time, 58 (7.2%) cysts were resected and of those, 11 had high grade dysplasia (HGD) or invasive cancer. Three additional patients had unresectable cancer for a total rate of malignancy of 1.7%. Predictors of development of malignancy included an increase in size ≥2.5 mm/year (HR = 29.54, 95% CI: 9.39-92.91, P < 0.001) and the development of worrisome features (HR = 9.17, 95% CI: 2.99-28.10, P = 0.001). Comparison of parametric survival models suggested that the risk of malignancy decreased after three years of surveillance and was lower than 0.2% after five years. CONCLUSIONS: Pancreatic cysts <15  mm at the time of diagnosis have a very low risk of malignant transformation. Our findings indicate the risk decreases over time. Size increase of ≥2.5 mm/year is the strongest predictor of malignancy.

International consensus guidelines for surgery and the timing of intervention in chronic pancreatitis.

BACKGROUND/OBJECTIVES: Chronic pancreatitis (CP) is a complex inflammatory disease with pain as the predominant symptom. Pain relief can be achieved using invasive interventions such as endoscopy and surgery. This paper is part of the international consensus guidelines on CP and presents the consensus guideline for surgery and timing of intervention in CP. METHODS: An international working group with 15 experts on CP surgery from the major pancreas societies (IAP, APA, JPS, and EPC) evaluated 20 statements generated from evidence on 5 questions deemed to be the most clinically relevant in CP. The Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach was used to evaluate the level of evidence available for each statement. To determine the level of agreement, the working group voted on the 20 statements for strength of agreement, using a nine-point Likert scale in order to calculate Cronbach's alpha reliability coefficient. RESULTS: Strong consensus was obtained for the following statements: Surgery in CP is indicated as treatment of intractable pain and local complications of adjacent organs, and in case of suspicion of malignant (cystic) lesion; Early surgery is favored over surgery in a more advanced stage of disease to achieve optimal long-term pain relief; In patients with an enlarged pancreatic head, a combined drainage and resection procedure, such as the Frey, Beger, and Berne procedure, may be the treatment of choice; Pancreaticoduodenectomy is the most suitable surgical option for patients with groove pancreatitis; The risk of pancreatic carcinoma in patients with CP is too low (2% in 10 year) to recommend active screening or prophylactic surgery; Patients with hereditary CP have such a high risk of pancreatic cancer that prophylactic resection can be considered (lifetime risk of 40-55%). Weak agreement for procedure choice in patients with dilated duct and normal size pancreatic head: both the extended lateral pancreaticojejunostomy and Frey procedure seems to provide equivalent pain control in patients. CONCLUSIONS: This international expert consensus guideline provides evidenced-based statements concerning key aspects in surgery and timing of intervention in CP. It is meant to guide clinical practitioners and surgeons in the treatment of patients with CP.

Diagnosis and management of pancreatic cystic neoplasms: current evidence and guidelines.

Pancreatic cystic neoplasms (PCN) are a heterogeneous group of pancreatic cysts that include intraductal papillary mucinous neoplasms, mucinous cystic neoplasms, serous cystic neoplasms and other rare cystic lesions, all with different biological behaviours and variable risk of progression to malignancy. As more pancreatic cysts are incidentally discovered on routine cross-sectional imaging, optimal surveillance for patients with PCN is becoming an increasingly common clinical problem, highlighting the need to balance cancer prevention with the risk of (surgical) overtreatment. This Review summarizes the latest developments in the diagnosis and management of PCN, including the quality of available evidence. Also discussed are the most important differences between the PCN guidelines from the American Gastroenterological Association, the International Association of Pancreatology and the European Study Group on Cystic Tumours of the Pancreas, including diagnostic and follow-up strategies and indications for surgery. Finally, new developments in the management of patients with PCN are addressed.

Mucinous nonneoplastic cyst of the pancreas penetrates the colon causing infection: a case report.

BACKGROUND: Mucinous nonneoplastic cyst of the pancreas is a rare disease defined as a cystic lesion lined with mucinous epithelium, supported by hypocellular stroma and not communicating with the pancreatic ducts. Mucinous nonneoplastic cyst of the pancreas has no malignant potential and does not require surgical resection or surveillance. However, its preoperative differentiation from other cystic lesions of the pancreas is difficult because of several overlapping clinical, radiological, and biochemical features. We report a rare case of large mucinous nonneoplastic cyst of the pancreas in which surgery was required due to infection and the possibility of malignancy. CASE PRESENTATION: A 75-year-old Japanese man was found to have a pancreatic cyst in 2006 while undergoing postoperative evaluation for colon cancer. In 2015, the cyst ruptured, and it was treated conservatively. In 2017, he fell down on a road with a fever of 40 °C and was transported emergently to a nearby hospital. Enhanced computed tomography revealed a cystic lesion in the body of the pancreas measuring 119 mm × 100 mm and an adjacent left renal cyst measuring 63 mm in diameter. The wall of the pancreatic cyst was thickened. Magnetic resonance imaging demonstrated a liquid surface in the pancreatic cyst. Pancreatic cyst infection was diagnosed as the source of infection. However, identification of the organism was difficult. Furthermore, due to the increase in the size and wall thickness of the cyst, it was unclear whether the cystic mass was neoplastic with malignant potential. For these reasons, the patient underwent distal pancreatectomy and splenectomy with deroofing of the left renal cyst. Intraoperatively, the pancreatic cyst adhered to the descending colon, and partial resection of the colon was added. Pathologic analysis of the resected cyst demonstrated a simple cyst lined by mucinous epithelium. There was no underlying stromal condensation or epithelial dysplasia, and communication with the native pancreatic ducts was not observed. Based on the operative and histological findings, a final diagnosis of mucinous nonneoplastic cyst of the pancreas with colonic communication was made. The colonic fistula was presumed to be the source of infection. CONCLUSION: Mucinous nonneoplastic cyst of the pancreas is generally benign and requires little follow-up, but large cysts may penetrate other organs and cause severe complications.

A pancreatic mucinous cystic neoplasm undergoing intriguing morphological changes over time and associated with recurrent pancreatitis: A case report.

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.

[Complex treatment of patient with chronic pancreatitis]. / Kompleksnoe lechenie bol'nogo khronicheskim pankreatitom.

Chronic pancreatitis complicated by pancreatic duct stones and pancreatic hypertension, post-necrotic cysts and fistulas requires surgical treatment. There are various procedures from minimally invasive to advanced pancreatectomy. Endoscopic interventions and radical open surgery allow to achieve good results. Successful complex treatment of patient with chronic pancreatitis is presented in this report.

[Diagnosis and treatment of pancreatic neuroendocrine neoplasmas in Von Hippel-Lindau syndrome].

Von Hippel-Lindau(VHL) syndrome is a rare autosomal dominant hereditary disease, and pancreas is one of the frequently involved intra-abdominal organs, including simple pancreatic cysts, pancreatic serous cystadenomas and neuroendocrine neoplasmas. Most of the VHL-related pancreatic neuroendocrine neoplasmas (VHL-pNEN)were non-functional, but they still have a tendency to be malignant. Treatment options for VHL-pNEN include regular follow-up, surgical resection, and medication therapy. When compared with sporadic pNEN, the malignant degree of VHL-pNEN is lower, with a better prognosis, so the surgical treatment should be carefully considered. The indications of surgery for VHL-pNEN include big primary lesions (≥3 cm), fast tumor doubling time (<500 days), VHL gene mutation on exon 3, malignant manifestations on imaging findings, and functional pNEN lesions. The function-preserving approach should be performed to keep the functional pancreatic parenchyma as much as possible. Even for patients with a late stage malignancy that cannot be radically resected, active medication therapy may still lead to a long-term survival.