IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 þ plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.

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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.

Objective To define how patients with pancreatic cysts are being diagnosed and treated. Design Retrospective case series. Setting University-affiliated tertiary care center. Patients Four hundred one patients evaluated in the Department of Surgery between January 2004 and December 2007. Main Outcome Measures Clinical management, histological diagnosis, and results of surveillance. Results Pancreatic cysts were incidentally discovered in 71% (284 of 401) of patients. There was no statistically significant difference in age (60.4 vs 63.1 years; P=.10), cyst size (31 vs 27 mm; P=.12), or histological diagnosis between symptomatic patients and patients with incidentally discovered cysts. Whereas the majority of symptomatic patients had their cystic neoplasms resected on diagnosis, 50% (142 of 284) of incidentally discovered cysts were initially managed nonoperatively. Of the patients who were managed with surveillance, 13 (8%) subsequently underwent resection after a median of 2.1 years because of an increase in cyst size, development of symptoms, increasing tumor markers, worrisome endoscopic ultrasonography findings, or patient anxiety. The most common diagnosis among resected lesions was either main-duct intraductal papillary mucinous neoplasm (25%) or branch-duct intraductal papillary mucinous neoplasm (23%). Invasive cancer was found in 29 of 256 (11%) resected cystic neoplasms, 9 of which were incidentally discovered, and in 7% (1 of 13) of patients who underwent watchful waiting prior to resection. Conclusions Incidentally discovered pancreatic cystic neoplasms composed 71% of our series, of which 50% were immediately resected. Subsequent morphologic changes or development of symptoms prompted an operation in 8% of patients after a period of surveillance. Invasive malignancy was present in 11% of all resected specimens but in 38% of main-duct intraductal papillary mucinous neoplasms.

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Objective Evaluate the value of cytology relative to imaging features in risk assessment for malignancy as defined in the Sendai Guidelines. Background The Sendai Guidelines list symptoms, cyst size > 30mm, dilated main pancreatic duct (MPD) > 6mm, mural nodule (MN) and “positive” cytology as high risk stigmata for malignancy warranting surgical triage. Methods We reviewed clinical, radiological and cytological data of 112 patients with histologically confirmed mucinous cysts of the pancreas evaluated in a single tertiary medical center. Cytology slides were blindly re-reviewed and epithelial cells grouped as either benign or high grade atypia (HGA) [≥ high grade dysplasia]. Histologically, neoplasms were grouped as benign (low-grade and moderate dysplasia) and malignant (in-situ and invasive carcinoma). Performance characteristics of cytology relative to other risk factors were evaluated. Results Dilated MPD, MN and HGA were independent predictors of malignancy (p<0.0001), but not symptoms (p=.29) or cyst size > 30 mm (p=.51). HGA was the most sensitive predictor of malignancy in all cysts (72%) and in small (≤ 30 mm) branch-duct IPMN (67%) while also being specific (85 and 88% respectively). MN and dilated MPD were highly specific (>90%), but insensitive (39–44%). Cytology detected 30% more cancers in small cysts than dilated MPD or MN and half of the cancers without either of these high risk imaging features. Conclusions Cytology adds value to the radiological assessment of predicting malignancy in mucinous cysts, particularly in small branch-duct IPMN.

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