Nisha I. Sainani

Subclassification of Autoimmune Pancreatitis: A Histologic Classification With Clinical Significance

Autoimmune pancreatitis (AIP) is a chronic inflammatory disease of the pancreas. Examination of pancreatic resection specimens from patients with AIP has shown that there are 2 subclasses of this disease. However, there is no widely accepted pathologic classification scheme and the clinical significance of such a classification remains to be established. In this study, we revisited the subclassification of AIP and examine whether this provides clinically and prognostically meaningful information. We evaluated 29 pancreatic resection specimens from patients with AIP. Demographic, clinical, and imaging data were recorded, as was evidence of extrapancreatic manifestations. In addition to a detailed and semiquantitative histologic evaluation, immunohistochemistry for IgG4 was performed on pancreatic and extrapancreatic tissues. We also evaluated 48 consecutive cases of chronic pancreatitis, not otherwise specified. The resected specimens could readily be subclassified into 2 subtypes: type 1 (n=11) and type 2 (n=18). In comparison with patients with type 2 disease, patients with type 1 disease were significantly more likely to be males (P=0.09), older (P=0.02), and present with jaundice (P=0.01), and less likely to be associated with abdominal pain (P=0.04). On imaging, the pancreatic tail cut-off sign was exclusively seen in patients with type 2 disease (4 of 10 cases). Hypercellular inflamed interlobular stroma was unique to type 1 pattern (91%), whereas significant ductal injury in the form of microabscesses and ductal ulceration was almost exclusively seen in type 2 pattern (78%). Eight of 10 patients with a type 1 pattern had evidence of a systemic disease. Three patients with type 2 disease had recurrent episodes of pancreatitis after their pancreatic resection. In comparison with the cohort of chronic pancreatitis, not otherwise specified, type 2 AIP cases were less likely to be associated with a history of alcohol abuse, and showed significantly more foci of periductal inflammation and neutrophilic microabscesses. Our review of pancreatic resection specimens shows 2 histologically distinct forms of AIP. Our data support the concept that type 1 AIP is a systemic disease and is the pancreatic manifestation of IgG4-related systemic disease. Type 2 disease is confined to the pancreas. The intensity of the periductal inflammatory infiltrate and the presence of ductal neutrophilic abscesses are features that assist in distinguishing type 2 AIP from chronic pancreatitis, not otherwise specified. Although imperfect, clinical and imaging features may help distinguish the 2 subtypes of AIP. On the basis of these significant differences between the 2 types of AIP, we advocate the position that all subsequent studies attempt to substratify their patients into these 2 groups.

IgG4-associated cholangitis: a comparative histological and immunophenotypic study with primary sclerosing cholangitis on liver biopsy material

IgG4-associated cholangitis is a steroid-responsive hepatobiliary inflammatory condition associated with autoimmune pancreatitis that clinically and radiologically mimics primary sclerosing cholangitis. In this study, we conducted a morphological and immunohistochemical analysis of liver material obtained from individuals with IgG4-associated cholangitis, and compared these with well-characterized cases of primary sclerosing cholangitis. The study group consisted of 10 patients (9 biopsy and 1 hepatectomy case) with IgG4-associated cholangitis and 17 patients with primary sclerosing cholangitis (16 needle biopsy and 1 hepatectomy case). All patients with IgG4-associated cholangitis had pancreatic involvement as well, and six pancreatectomy samples revealed characteristic histopathological features of autoimmune pancreatitis. Primary sclerosing cholangitis cases were defined by the presence of a characteristic ERCP appearance. Clinical, pathological, radiological, and follow-up data were recorded for all cases. Portal and periportal inflammation was graded according to Ishaks guidelines. Immunohistochemical stains for IgG and IgG4 were performed. The cohort of patients with IgG4-associated cholangitis (mean age: 63 years) was older than individuals with primary sclerosing cholangitis (mean age: 44 years). Seven of these cases showed intrahepatic biliary strictures. IgG4-associated cholangitis liver samples showed higher portal (P=0.06) and lobular (P=0.009) inflammatory scores. Microscopic portal-based fibro-inflammatory nodules that were composed of fibroblasts, plasma cells, lymphocytes, and eosinophils were exclusively observed in five of the IgG4-associated cholangitis cases (50%). More than 10 IgG4-positive plasma cells per HPF (high power field) were observed in 6 of the IgG4-associated cholangitis cases (mean: 60, range: 0–140 per HPF), whereas all primary sclerosing cholangitis cases showed significantly lesser numbers (mean: 0.08, range: 0–1 per HPF). On a liver biopsy, the histological features of IgG4-associated cholangitis may be distinctive, and in conjunction with IgG4 immunohistochemical stain, may help distinguish this disease from primary sclerosing cholangitis.

Comparative performance of MDCT and MRI with MR cholangiopancreatography in characterizing small pancreatic cysts.

OBJECTIVE The objective of our study was to compare MDCT with MRI-MR cholangiopancreatography (MRCP) in characterizing small pancreatic cysts (< or = 3 cm) and predicting aggressiveness. MATERIALS AND METHODS In a retrospective analysis, contrast-enhanced MDCT and MRI examinations of 30 patients with 38 pathologically confirmed small pancreatic cysts were reviewed. MDCT and MRCP studies were independently evaluated by two readers for cyst morphology, cyst characterization, and prediction of lesion aggressiveness, which included lesions with moderate-grade dysplasia, high-grade dysplasia (carcinoma in situ), and invasive carcinomas. The sensitivity of MDCT and MRI for the detection of each morphologic feature, accuracy for cyst characterization, and predictive values for aggressiveness were calculated. RESULTS Of 38 lesions, 14 were side-branch intraductal papillary mucinous neoplasms (IPMNs), 12 mixed IPMNs, six mucinous cystic neoplasms, and six nonneoplastic cysts. On histopathology, 26 lesions were nonaggressive (six nonneoplastic cysts, six benign mucinous cystic neoplasms, 14 low-grade dysplasias in IPMNs), whereas 12 lesions revealed aggressive biology (eight moderate-grade dysplasias, four high-grade dysplasias in IPMNs). The sensitivity of MRCP for the detection of morphologic features was better than that of MDCT, but the differences were not statistically significant (p = 0.25-1). Interreader agreement and MDCT-MRI agreement for morphologic features were good to perfect (kappa = 0.7-1). The accuracy of MDCT and MRI was higher in classifying cysts as mucinous or nonmucinous than in determining a specific diagnosis (71-84.2% vs 39.5-44.7%, respectively), whereas the accuracy of the two techniques in characterizing cysts into nonaggressive and aggressive categories was similar (MDCT vs MRI, 75-78% vs 78-86%, respectively; p > 0.05). CONCLUSION MRI enables more confident assessment of the morphology of small cysts than MDCT, but the accuracy of the two imaging techniques for cyst characterization is comparable. MDCT and MRI have high accuracy in classifying cysts into mucinous and nonmucinous categories and perform similarly in estimating histologic aggressiveness.

Rethinking Ormond's disease: "idiopathic" retroperitoneal fibrosis in the era of IgG4-related disease.

AbstractIdiopathic retroperitoneal fibrosis (RPF) is a periaortic sclerotic disease that encases adjacent retroperitoneal structures, particularly the ureters. A subset of idiopathic RPF cases can be associated with IgG4-related disease, but the frequency of this association is not clear. We selected 23 cases of idiopathic RPF and identified IgG4-related RPF cases based on the presence of IgG4+ plasma cells in the tissue, using an IgG4/IgG ratio cutoff of >40%. We then compared the IgG4-related RPF patients and the non-IgG4-related RPF patients in terms of both the presence of histopathologic features typical of IgG4-related disease and the simultaneous occurrence (or history) of other organ manifestations typical of IgG4-related disease. The IgG4-related RPF and non-IgG4-related RPF groups were also analyzed in terms of clinical, laboratory, and radiologic features and treatment review.We identified 13 cases of IgG4-related RPF (57% of the total cohort). The distinguishing features of IgG4-related RPF were histopathologic and extra-organ manifestations of IgG4-related disease. The IgG4-related RPF patients were statistically more likely than non-IgG4-related RPF patients to have retroperitoneal biopsies showing lymphoplasmacytic infiltrate (p = 0.006), storiform fibrosis (p = 0.006), or tissue eosinophilia (p = 0.0002). Demographics of the 2 groups, including a middle-aged, male predominance (mean age, 58 yr; 73% male), were similar.IgG4-related disease accounts for a substantial percentage of patients with “idiopathic” RPF. Histopathologic features such as storiform fibrosis, obliterative phlebitis, and tissue eosinophilia are critical to identifying this disease association. Extraretroperitoneal manifestations of IgG4-related disease are also often present among patients with IgG4-related RPF. Elevated IgG4/total IgG ratios in tissue biopsies are more useful than the number of IgG4+ plasma cells per high-power field in cases of RPF that are highly fibrotic.

Cholangiocarcinoma: Current and Novel Imaging Techniques

The radiologic manifestations of cholangiocarcinomas are extremely diverse, since these tumors vary greatly in location, growth pattern, and histologic type. Familiarity with the imaging manifestations of cholangiocarcinomas is important for accurate detection and characterization of these tumors and assessment of resectability. Advances in imaging techniques have led to the availability of an array of modalities that, used independently or in combination, can aid in the accurate diagnosis and evaluation of cholangiocarcinomas in preparation for advanced surgical procedures and treatment planning. Response to novel targeted therapies can also be assessed with newer imaging tools. Hence, knowledge of current and emerging imaging applications is essential for correct diagnosis and appropriate management of these tumors.

Imaging after percutaneous radiofrequency ablation of hepatic tumors: Part 2, Abnormal findings.

OBJECTIVE The purpose of this article is to review the spectrum of early and delayed abnormal cross-sectional imaging findings after radiofrequency ablation (RFA) of hepatic tumors. CONCLUSION Recognition of abnormal post-RFA imaging findings and differentiation of abnormal findings from normal postprocedural changes are important for diagnostic and interventional radiologists. Early identification of residual or recurrent disease and complications can facilitate timely retreatment, management, and follow-up care.

Multidisciplinary Approach to Diagnosis and Management of Intraductal Papillary Mucinous Neoplasms of the Pancreas

Intraductal papillary mucinous neoplasms have gained recognition in recent years as premalignant precursors to pancreatic cancer that enable early detection and often are found incidentally at imaging. Accurate diagnosis and optimal, finely tuned management of these lesions are important and require collaboration across various disciplines, including radiology, endoscopy, surgery, and pathology. Several imaging modalities can visualize these lesions adequately, each with specific advantages and disadvantages. Multidetector computed tomography and magnetic resonance cholangiopancreatography are generally the first-line imaging modalities; endoscopic imaging such as endoscopic ultrasound and endoscopic retrograde cholangiopancreatography are beneficial when the former 2 modalities are equivocal. Surgical candidates generally include patients with main duct lesions or branch duct lesions greater than 3 cm or any possessing a solid component. A management algorithm indicating when surgery should be pursued is proposed. For nonsurgical and postsurgical patients, follow-up management is important to monitor growth and recurrence, and risks from repeated radiation exposure should be taken into account. Furthermore, issues of multifocality and increased predisposition of the pancreas to ductal adenocarcinoma must be addressed at follow-up evaluation. A follow-up management algorithm also is proposed in this review.

Prospective Evaluation of Reader Performance on MDCT in Characterization of Cystic Pancreatic Lesions and Prediction of Cyst Biologic Aggressiveness

OBJECTIVE Our objective was to evaluate the accuracy of MDCT features of pancreatic cystic lesions in cyst characterization and in predicting cyst biologic aggressiveness. SUBJECTS AND METHODS In this prospective study, 114 patients (40 men and 74 women; age range, 23-89 years) with 130 cystic lesions (size range, 31-160 mm) in the pancreas underwent contrast-enhanced dual-phase (n = 92) and portal phase (n = 22) examinations with 16- or 64-MDCT scanners. Using defined morphologic features of cystic lesions on MDCT, two readers performed blinded evaluations for cystic characterization and predicting biologic aggressiveness (invasive lesions, carcinoma in situ, and moderate grade dysplasias) before pancreatic surgery. Receiver operating characteristic analysis was performed to assess the accuracy of MDCT using pathologic evaluation of the surgical specimen as a reference standard. RESULTS On the basis of MDCT features, the radiologic accuracy (reader 1 and reader 2) for stratifying lesions into mucinous and nonmucinous subtypes was 85% and 82% and for recognizing cysts with aggressive biology was 86% and 85%, respectively. Predictive values of MDCT were superior for lesions > 30 mm and nonmucinous lesions. Features favoring aggressive biology were main pancreatic duct dilation > 10 mm (p < 0.0001), biliary obstruction (p=0.01), mural nodule (p < 0.0001), main-duct intraductal papillary mucinous neoplasm (p < 0.0001), and advanced age (p = 0.0001). Sensitivity of detecting morphologic features was higher with the dual-phase pancreatic protocol CT. CONCLUSION Morphologic features of pancreatic cystic lesions on MDCT allow reliable characterization into mucinous and nonmucinous subtypes and enable prediction of biologic aggressiveness.

Impact of a Structured Report Template on the Quality of MRI Reports for Rectal Cancer Staging

OBJECTIVE The purpose of this study is to assess the impact of implementing a structured report template on the quality of MRI reports for rectal cancer staging. MATERIALS AND METHODS After excluding examinations performed after surgery or neoadjuvant therapy, we analyzed all rectal cancer staging MRI reports finalized at an academic medical center 12 months before and after an intervention consisting of implementing a structured report template integrated into the institutions speech recognition system. The primary outcome measure was the quality of rectal cancer staging MRI reports classified as optimal, satisfactory, or unsatisfactory, on the basis of the documentation of 14 quality measures predefined by a consensus of the institutions abdominal radiology subspecialists. Chi-square and t tests were used to assess differences in report quality and documentation of each discrete quality measure before and after the intervention. RESULTS The study cohort included 106 MRI reports from 104 patients (mean age, 60 years; 58.5% male); 52 (49.1%) of the reports were completed before implementation of the structured report template. After implementation, the proportion of total reports classified as optimal or satisfactory increased from 38.5% (20/52) to 70.4% (38/54) (p = 0.0010). No reports generated before the intervention were classified as optimal, whereas 40.7% (22/54) of reports were classified as optimal after the intervention. CONCLUSION Implementation and voluntary use of a structured report template improved the quality of MRI reports for rectal cancer staging compared with free-text format.

The challenging image-guided abdominal mass biopsy: established and emerging techniques ‘if you can see it, you can biopsy it’

Image-guided percutaneous biopsy of abdominal masses is among the most commonly performed procedures in interventional radiology. While most abdominal masses are readily amenable to percutaneous biopsy, some may be technically challenging for a number of reasons. Low lesion conspicuity, small size, overlying or intervening structures, motion, such as that due to respiration, are some of the factors that can influence the ability and ultimately the success of an abdominal biopsy. Various techniques or technologies, such as choice of imaging modality, use of intravenous contrast and anatomic landmarks, patient positioning, organ displacement or trans-organ approach, angling CT gantry, triangulation method, real-time guidance with CT fluoroscopy or ultrasound, sedation or breath-hold, pre-procedural image fusion, electromagnetic tracking, and others, when used singularly or in combination, can overcome these challenges to facilitate needle placement in abdominal masses that otherwise would be considered not amenable to percutaneous biopsy. Familiarity and awareness of these techniques allows the interventional radiologist to expand the use of percutaneous biopsy in clinical practice, and help choose the most appropriate technique for a particular patient.