Riccardo Negrelli

Application of international consensus diagnostic criteria to an Italian series of autoimmune pancreatitis

Background International consensus diagnostic criteria (ICDC) have been proposed to classify autoimmune pancreatitis (AIP) in type 1, type 2, or not otherwise specified. Objective Aim was to apply the ICDC to an Italian series of patients to evaluate the incidence and clinical profiles among different subtypes of AIP. Methods we re-evaluated and classified 92 patients diagnosed by Verona criteria, according to the ICDC. Results Out of 92 patients, 59 (64%) were diagnosed as type 1, 17 (18%) as type 2, and 15 (16%) as not otherwise specified according to the ICDC. A significant difference between type 1 and type 2 were found for age (54.5 ± 14.5 vs. 34.4 ± 13.9 respectively; p < 0.0001), male sex (76 vs. 47%; p = 0.007), jaundice (66 vs. 18%; p = 0.002) and acute pancreatitis (9 vs. 47%; p < 0.0001), elevated serum IgG4 levels (85 vs. 7%; p < 0.0001), inflammatory bowel disease (8 vs. 82%; < 0.0001), and relapse of the disease (34 vs. 6%; p = 0.058). Imaging and response to steroids in the not-otherwise-specified group were similar to type 1 and 2. Conclusions Type 1 has a different clinical profile from type 2 autoimmune pancreatitis. The not-otherwise-specified group has peculiar clinical features which are shared both with type 1 or type 2 groups.

Detection and localization of deep endometriosis by means of MRI and correlation with the ENZIAN score

PURPOSE To determine the accuracy of ENZIAN score, as detected on MR imaging, compared to surgical-pathologic findings. MATERIALS AND METHODS This retrospective study was approved by the investigational review board and the requirement for informed patient consent was waived. 115 patients were included according to following criteria: tubo-ovarian and/or deep endometriosis suspected at physical examination and transvaginal ultrasound; availability of MR examination; histopathological results from laparoscopic or surgical treatment. EXCLUSION CRITERIA lack of available MR examination, and/or (b) lack of a definitive histopathological results. Histopathological findings from bioptic specimens obtained during laparoscopic or laparotomic treatment were considered as reference standard. For all detected lesions a score according to ENZIAN score (revised 2010) was assigned both for MRI and histopathological findings. By comparing MRI-ENZIAN score and histopathological-ENZIAN score the overall sensitivity, specificity, accuracy, positive and negative predictive values in relation to presence/absence of deep endometriosis in each patient were calculated. k-Cohen to evaluate the degree of concordance between MRI-ENZIAN score and histopathological-ENZIAN score was also measured. Moreover the sensitivity, specificity, accuracy, positive and negative predictive values for each specific localization provided by ENZIAN score were also calculated. RESULTS At histopathology, the diagnosis of deep endometriosis was confirmed in 82/115 (71.3%) patients. The sensitivity, specificity, accuracy, PPV and NPV of MRI were 94%, 97%, 95%, 99%, 86%, respectively. The highest accuracy was for adenomyosis (100%) and endometriosis of utero-sacral ligaments (USLs) (98%), slightly lower for vagina-rectovaginal septum an colo-rectal walls (96%), and the lowest for bladder endometriosis (92%). The concordance between histopathological and MRI ENZIAN score was excellent (k=0.824); in particular it was 0.812 for lesions in vagina-rectovaginal space, 0.890 for lesions in USL, 0.822 for lesions in rectum-sigmoid colon, 1.000 for uterine adenomyosis, and 0.367 for lesions located in the bladder wall. CONCLUSION MRI correlates with the ENZIAN score and has an accuracy of 95% in the detection and localization of deep endometriosis, allowing to minimize false negative results (4%) in patients with deep endometriosis and to obtain a correct preoperative staging.

Multifocal branch-duct intraductal papillary mucinous neoplasms (IPMNs) of the pancreas: magnetic resonance (MR) imaging pattern and evolution over time

PurposeThe aim of our study was to follow the evolution over time of multifocal intraductal papillary mucinous neoplasms (IPMN) of the pancreatic duct side branches by means of magnetic resonance imaging (MRI).Materials and methodsA total of 155 patients with multifocal IPMN of the side branches were examined with MRI and MR cholangiopancreatography (MRI/MRCP). Inclusion criteria were patients with ≥2 dilated side branches involving any site of the parenchyma; presence of communication with the main pancreatic duct and previous investigations by MRI/MRCP within at least six months. Median follow-up was 25.8 months (range, 12–217). Patients with a follow-up period shorter than 12 months (n=33) and those with a diagnosis of multifocal IPMN of the side branches without any follow-up (n=14) were excluded from the study. The final study population thus comprised 108 patients. A double, quantitative and qualitative, analysis was carried out. The quantitative image analysis included: number of dilated side branches in the head-uncinate process and body-tail; maximum diameter of lesions in the head-uncinate process; maximum diameter in the body-tail; maximum diameter of the main pancreatic duct in the head and body-tail. The qualitative image analysis included: presence of malformations or anatomical variants of the pancreatic ductal system; site of the lesions (head-uncinate process, body-tail, ubiquitous, bridge morphology); presence of gravity-dependent intraluminal filling defects; presence of enhancing mural nodules.ResultsAt diagnosis, the mean number of cystic lesions of the side branches was 7.09. The mean diameter of the cystic lesions was 13.7 mm. The mean diameter of the main pancreatic duct was 3.6 mm. At follow-up, the mean number of cystic lesions was 7.76. The mean diameter of the cystic lesions was 13.9 mm. The mean diameter of the main pancreatic duct was 3.7 mm. Intraluminal filling defects in the side branches were seen in 18/108 patients (16.6%); enhancing mural nodules were seen in 3/108 patients (2.7%).ConclusionsMultifocal IPMN of the branch ducts shows a very slow growth and evolution over time. In our study, only 3/108 patients showed mural nodules which, however, did not require any surgical procedure, indicating that careful nonoperative management may be safe and effective in asymptomatic patients.RiassuntoObiettivoL’obiettivo che il nostro studio si propone è quello di seguire l’evoluzione nel tempo delle neoplasie multifocali mucinose intraduttali papillari (IPMN) dei rami collaterali per mezzo della risonanza magnetica (RM).Materiali e metodiSono stati valutati 155 pazienti con IPMN multifocali dei dotti secondari esaminati con RM e con colangiopancreatografia RM (CPRM). Criteri di inclusione: pazienti con ≥2 rami collaterali dilatati che coinvolgono qualunque sito del parenchima pancreatico; presenza di comunicazione con il dotto pancreatico principale, ≥2 esami precedenti RM/CPRM a distanza di almeno sei mesi. La mediana del monitoraggio è stata 25,8 (range 12–217) mesi. Criteri di esclusione: pazienti con un periodo di osservazione inferiore ai 12 mesi (n=33), ed i pazienti con diagnosi di IPMN multifocale dei dotti di II ordine che non hanno un follow-up (n=14). La popolazione considerata è quindi di 108 pazienti. è stata effettuata una duplice analisi, quantitativa e qualitativa. L’analisi quantitativa comprendeva: numero delle ectasie cistiche dei dotti collaterali nella testa-processo uncinato e nel corpo-coda; diametro massimo delle lesioni nella testa-processo-uncinato; diametro massimo nel corpocoda, diametro massimo del dotto pancreatico principale nella testa e nel corpo-coda. L’analisi qualitativa comprendeva: presenza/assenza di malformazioni/varianti anatomiche del sistema duttale pancreatico, localizzazione delle lesioni nel parenchima pancreatico, presenza di difetti endoluminali declivi, presenza di noduli parietali con impregnazione di mezzo di contrasto.RisultatiAlla diagnosi il numero medio di ectasie cistiche dei rami collaterali è stato 7,09. Il diametro medio delle ectasie cistiche era di 13,7 mm. Il diametro medio del dotto pancreatico principale era di 3,6 mm. Al follow-up il numero medio di ectasie cistiche era di 7,76. Il diametro medio delle lesioni cistiche era di 13,9. Il diametro medio del dotto pancreatico principale era di 3,7 mm. In 18/108 pazienti (16,6%) sono stati osservati difetti di riempimento intraluminali nei dotti pancreatici secondari, mentre sono stati riscontrati noduli murali in 3/108 pazienti (2,7%).ConclusioniGli IPMN multifocali dei dotti pancreatici secondari mostrano una crescita molto lenta. Nel nostro studio solo 3/108 pazienti hanno mostrato noduli murali, che comunque non sono stati sottoposti ad intervento chirurgico.

Retrospective comparison between preoperative diagnosis by International Consensus Diagnostic Criteria and histological diagnosis in patients with focal autoimmune pancreatitis who underwent surgery with suspicion of cancer

Objective The objective of this study was to compare the preoperative diagnosis by International Consensus Diagnostic Criteria (ICDC) with histological diagnosis in patients with focal autoimmune pancreatitis (AIP) who underwent surgery. Methods Thirty patients (type 1 AIP in 23 and type 2 AIP in 7) with a diagnosis of AIP based on histology of surgical specimens were classified according to ICDC based on their preoperative data. Results Pancreatic core biopsies and diagnostic steroid trial were not preoperatively performed in any of the patients. Based on preoperative data, ICDC diagnosed 6 patients (20%) as having type 1 AIP and 24 (80%) as probable AIP. Assuming all patients had responded to a steroid trial preoperatively, ICDC would have diagnosed 8 patients (27%) as having type 1 AIP, 4 (13%) as type 2 AIP, 10 as AIP–not otherwise specified (33%), and 8 (27%) as probable AIP. In the hypothetical situation, 7 of 8 type 1 AIP patients and 3 of 3 type 2 AIP patients would have been classified into the correct subtype of AIP. Conclusions A steroid trial enhances the possibility of correctly diagnosing AIP by ICDC despite the lack of histology. However, some patients cannot be diagnosed as having AIP or be classified into the correct subtype without histology.

Pancreatic duct stenosis: Differential diagnosis between malignant and benign conditions at secretin-enhanced MRCP

PURPOSE To define imaging criteria of benign and malignant nature in patients with main pancreatic duct (MPD) stenosis. MATERIALS-METHODS S-MRCPs of 35 patients with pancreatitis and 14 with adenocarcinoma were evaluated. RESULTS Adenocarcinoma caused higher prevalence of complete stenosis (14/14-100% vs 17/35-49%), dilated side-branches (14/14-100% vs 18/35-51%) and lower prevalence of duct-penetrating sign (0/14-0% vs 31/35-89%). The number of stenoses was higher in benign conditions (mean 1.4 Vs 1). Upstream MPD diameter was higher in cancer-induced stenoses (4.5 vs 2.9mm). CONCLUSIONS Single complete stenosis with dilated side branches, increased MPD caliber and absent duct-penetrating sign are suggestive of malignancy.

Clinical and Morphological Features of Paraduodenal Pancreatitis: An Italian Experience With 120 Patients

Objectives This study aimed to evaluate the clinical and radiological features and clinical outcomes of paraduodenal pancreatitis (PP). Methods A final diagnosis of PP was based on surgical specimens in resected patients and on imaging in nonoperated patients. Clinical, radiological, and pathological data were collected and reevaluated. Results We studied 120 patients, 97.5% of whom were drinkers and 97.5% were smokers. Symptoms at clinical onset were acute pancreatitis in 78 patients (65%) and continuous pain in 68 patients (55.8%). Other symptoms were vomiting (36.7%), weight loss (25.8%), and jaundice (11.7%). Cystic variant was diagnosed in 82 patients (68.0%), and solid variant was diagnosed in 38 patients (32.0%). Pure and diffuse forms were observed in 22 (18.3%) and 98 (81.7%) patients, respectively. Pancreatic calcifications were present at clinical onset in 5.0% of the patients and in 61.0% at the end of follow-up. Somatostatin analogs were used in 13 patients (10.8%), and 81 patients (67.0%) underwent surgery. Conclusions The clinical profile of PP was found to be middle-aged men who were heavy drinkers and smokers with painful pancreatitis and was associated with vomiting and weight loss. In nonresponders, alcohol withdrawal and medical therapy can be proposed as a first-line treatment, and surgery as a second-line treatment.

Surgery after FOLFIRINOX treatment for locally advanced and borderline resectable pancreatic cancer: increase in tumour attenuation on CT correlates with R0 resection

ObjectivesTo assess factors associated with radical resection (R0) of pancreatic ductal adenocarcinoma (PDAC) after induction treatment with FOLFIRINOX.MethodsPatients with either locally advanced (LA) and borderline resectable (BR) PDAC undergoing surgical exploration after FOLFIRINOX were retrospectively enrolled. Two pancreatic radiologists reviewed the CT blinded to the final outcome and assessed chemotherapy response and resectability. Patients were then divided into R0 resected (group A) and not resected/R1 resected (group B), which were compared.ResultsOf 59 patients included, 19 were defined as unresectable (32%), 33 borderline resectable (56%) and 7 resectable (12%) during the blind radiological evaluation after FOLFIRINOX. Once in a surgical setting, 27% were non-resectable, whereas 73% received surgical resection with a 70% R0 rate. Consequent sensitivity and specificity were 86% and 29%. At imaging review, significant decreases in longest tumour dimension were observed in both groups: from 32 mm (95% CI 15–55) to 21 (10–44) in group A and from 34 (18–70) to 26 (7–60) in group B, p < 0.05. However, a significant increase in tumour attenuation in all phases was only observed for R0 resected, from 52 HU (26–75) to 65 (35–92) in arterial phase (p < 0.001) and from 62 (36–96) to 78 (40–120) in the venous (p = 0.001).ConclusionAfter neoadjuvant FOLFIRINOX, CT predicted resectability with acceptable sensitivity but low specificity. The observation of increased tumour attenuation at CT scan after FOLFIRINOX treatment might represent a reliable predictor of R0 resection.Key Points• CT drives the assessment of PDAC resectability after FOLFIRINOX• CT predicts resectability with acceptable sensitivity but low specificity• Significant increase in tumour attenuation was only observed for R0 resected PDAC• Tumour attenuation after FOLFIRINOX represents a reliable predictor of R0 resection

Paraduodenal pancreatitis as a mimicker of pancreatic adenocarcinoma: MRI evaluation

PURPOSE To evaluate the MRI features of paraduodenal pancreatitis (PDP) and to define useful signs to differentiate PDP from pancreatic ductal adenocarcinoma (PDAC). MATERIAL AND METHODS We reviewed the MRI scans of 56 patients, 28 affected by PDP and 28 by PDAC, all pathologically proven. The following parameters were evaluated: signal intensity of the lesion on T1-, T2-WI, DWI (b800) and after contrast medium administration; presence of cysts; dilation of common hepatic duct and main pancreatic duct; focal thickening of the second portion of the duodenum; maximum diameter and volume of the lesion. RESULTS Both PDPs and PDACs were more frequently hypointense on T1-WI, iso-hyperintense on T2-WI, hypointense in the pancreatic phase and iso-hypointense in the venous phase (p>0.05); in the delayed phase most PDP were hyperintense (p=0.0031); on DWI 71.4% PDPs were isointense and all PDACs were hyperintense (p=0.0041). Cystic components were present in 85.7% PDPs (p=0.0011); double duct sign was present in 50% PDACs (p=0.0048); focal thickening of the duodenum was depicted in 89.3 PDPs (p=0.0012). PDPs were larger than PDACs (p=0.0003). CONCLUSION The most suggestive signs of PDP are: signal hyperintensity in the delayed phase, isointensity on DWI, presence of cysts, focal thickening of the duodenum and large size of the lesion.

Type 1 and type 2 autoimmune pancreatitis: is there any difference in MRI?

Purpose: The justification and optimisation of medical imaging employing ionizing radiation have been intensely discussed in recent years, particularly for computed tomography (CT). A key point in this discussion is the estimation of patient dose, which commonly employs radiation output metrics developed for quality assurance and no patient specific information. Such patient dose estimates are of limited value, and more refined methods needs to be promoted and provided to the community. Methods and Materials: AAPM Task Group 246 was formed in 2013, and in a joint venture with EFOMP charged with summarizing present methodology and DICOM information available for estimating patient dose with computed tomography.Results: The Joint Report of AAPM Task Group 246 and EFOMP is a comprehensive resource for the clinical medical physicist. The possibilities of patient specific dosimetry from the Computed Tomography Dose Index (CTDIvol), to the Size-Specific Dose Estimates (SSDE) and advanced Monte Carlo methods are discussed together with available DICOM information, as well as practical examples on how patient dose estimates can be achieved. The report also summarizes important factors contributing to the uncertainty in patient dose estimates and gives examples of achievable confidence intervals.Conclusion: The SSDE and Monte Carlo methods can together with detailed scanner, examination and patient specific DICOM information offer refined estimates of patient dose for justification and optimisation of CT examinations. Given the present robustness of available methods AAPM Task Group 246 and EFOMP recommend that all reports of patient dose should be accompanied by estimates of the associated uncertainty.

Su1312 Classifications of an Italian Series of Autoimmune Pancreatitis by International Consensus Diagnostic Criteria (ICDC)

Background and Aims: International Consensus Diagnostic Criteria (ICDC) for autoimmune pancreatitis (AIP) have been proposed to identify type 1 AIP, type 2 AIP, and AIP-not otherwise specified (AIP-NOS). To date, no study on incidence, clinical profiles and outcome in the groups classified according to ICDC has been reported. The aim of this study was to apply these criteria to an Italian series of patients to evaluate the incidence, clinical profiles, and outcome among these subtypes of AIP. Patients and Methods: We re-evaluated and classified 92 patients (16 operated patients and 76 non-operated patients) diagnosed as having AIP by Verona criteria, according to ICDC. Results: Out of 92 patients, 59 patients (64%) were diagnosed as type 1 AIP, 17 (18%) as type 2 AIP, and 15 (16%) as AIP-NOS according to ICDC. A patient showing a spontaneous remission was diagnosed as probable AIP (that fulfilled Verona criteria but not ICDC). Five type 1 AIP patients with ulcerative colitis fulfilled the diagnostic criteria of probable type 2 AIP as well. However, these patients were finally classified into type 1 AIP according to algorithm of ICDC. Type 1 AIP patients compared to type 2 AIP were older (54.5 ± 14.5 vs 34.4 ± 13.9; p ,0.0001) and different in the frequency of male sex (76% vs 47%; p=0.007), jaundice (66% vs 18%; p=0.002) and acute pancreatitis (9% vs 47%; p,0.0001) as initial symptom, elevated serum IgG4 levels (85% vs 7%; p,0.0001), inflammatory bowel disease (8% vs 82%; ,0.0001), and relapse of the disease (34% vs 0%; p=0.002). The clinical and epidemiological parameters in AIPNOS, such as mean age (45.7 ± 14.9), frequency of jaundice (47%) and acute pancreatitis (40%) as initial symptom, and relapse rate (20%), were intermediate between those of type 1 and type 2 AIP. Imaging and response to steroids in AIP-NOS were similar to those in two subtypes of AIP. CONCLUSIONS: Patients diagnosed as type 1 AIP by ICDC have different clinical profiles and outcome from those as type 2 AIP. Although AIP-NOS group seem to be composed of type 1 and type 2 AIP cases without typical clinical and serological features, we cannot exclude an overlap syndrome as a separate entity or as a possible evolution from type 2 AIP to type 1 AIP. ICDC may be unable to diagnose patients with spontaneous remission as having AIP.