Autoimmune pancreatitis: multidetector-row computed tomography (MDCT) and magnetic resonance (MR) findings in the Italian experience.

Multidetector-row computed tomography (MDCT) and magnetic resonance (MR) imaging are currently the most frequently performed imaging modalities for the study of pancreatic disease. In cases of suspected autoimmune pancreatitis (AIP), a dynamic quadriphasic (precontrast, contrast-enhanced pancreatic, venous and late phases) study is recommended in both techniques. In the diffuse form of autoimmune pancreatitis (DAIP), the pancreatic parenchyma shows diffuse enlargement and appears, during the MDCT and MR contrast-enhanced pancreatic phase, diffusely hypodense and hypointense, respectively, compared to the spleen because of lymphoplasmacytic infiltration and pancreatic fibrosis. During the venous phase of MDCT and MR imaging, the parenchyma appears hyperdense and hyperintense, respectively, in comparison to the pancreatic phase. In the delayed phase of both imaging modalities, it shows retention of contrast media. A "capsule-like rim" may be recognised as a peripancreatic MDCT hyperdense and MR hypointense halo in the T2-weighted images, compared to the parenchyma. DAIP must be differentiated from non-necrotizing acute pancreatitis (NNAP) and lymphoma since both diseases show diffuse enlargement of the pancreatic parenchyma. The differential diagnosis is clinically difficult, and dynamic contrast-enhanced MDCT has an important role. In the focal form of autoimmune pancreatitis (FAIP), the parenchyma shows segmental enlargement involving the head, the body-tail or the tail, with the same contrast pattern as the diffuse form on both modalities. FAIP needs to be differentiated from pancreatic adenocarcinoma to avoid unnecessary surgical procedures, since both diseases have similar clinical and imaging presentation. The differential diagnosis is clinically difficult, and dynamic contrast-enhanced MDCT and MR imaging both have an important role. MR cholangiopancreatography helps in the differential diagnosis. Furthermore, MDCT and MR imaging can identify the extrapancreatic manifestations of AIP, most commonly biliary, renal and retroperitoneal. Finally, in all cases of uncertain diagnosis, MDCT and/or MR follow-up after short-term treatment (2-3 weeks) with high-dose steroids can identify a significant reduction in size of the pancreatic parenchyma and, in FAIP, normalisation of the calibre of the upstream main pancreatic duct.

Spectrum of magnetic resonance imaging findings in pancreatic and other abdominal manifestations of Von Hippel-Lindau disease in a series of 23 patients: a pictorial review.

CONTEXT: Von Hippel Lindau disease is a rare autosomal dominantly inherited multisystem disorder characterized by development of benign and malignant tumors. The abdominal manifestation of the syndrome are protean. Magnetic resonance plays an important role in identification of abdominal abnormalities and follow-up of lesions. OBJECTIVE: To describe magnetic resonance imaging findings and patterns of pancreatic and other principal abdominal manifestations in a series of von Hippel-Lindau (VHL) disease patients and to review literature. METHODS: We retrospectively reviewed abdominal magnetic resonance studies performed in 23 patients (10 males, 13 females) diagnosed of VHL. RESULTS: In all examined patients abdominal involvement was present. The pancreatic imaging findings detected were: unilocular cystic lesions (6/23: 26.1%); serous cystadenomas (11/23: 47.8%), including diffuse lesions (8/23: 34.8%); solid neuroendocrine tumors (8/23: 34.8%); cystic neuroendocrine tumors (1/23: 4.3%). The renal findings detected were: simple renal cysts (18/23: 78.3%); complex renal cysts (13/23: 56.5%), including benign lesions (10/23: 43.5%) and malignant lesions (3/23: 13.0%); renal carcinomas (11/23: 47.8%) and 5 of these (45.5%) were multiple and bilateral. Five patients (21.7%) presented pheochromocytoma (4 of these were bilateral; 80.0%) and 1 patient (4.3%) presented cystadenoma of the epididymis. CONCLUSIONS: In VHL disease patients, magnetic resonance imaging plays an essential role in the identification of pancreatic and other abdominal lesions, in their follow-up, in the screening of asymptomatic gene carriers, and in their long-term surveillance.

Non-hyperfunctioning neuroendocrine tumours of the pancreas: MR imaging appearance and correlation with their biological behaviour.

OBJECTIVE: To describe MR imaging features of non-hyperfunctioning neuroendocrine pancreatic tumours by comparing them to histopathology and to determine the accuracy of MR imaging in predicting biological behaviour. MATERIALS AND METHODS: After institutional review board approval, we retrospectively reviewed 45 patients with pathologically proven NF-NET of the pancreas and ≥1 preoperative MR/MRCP examinations. Of the NF-NETS, 29/45 (64.4 %) were G1 and 16/45 (35.5 %) were G2. Image analysis included the lesion maximum diameter, vascular encasement, extrapancreatic spread, signal intensity on T1- and T2-weighted, contrast enhancement features, and presence of metastases. Tumour vessel density was calculated on the histological specimen using a grid. RESULTS: The median maximum diameter of NF-NETs was 20 mm (range 5-200 mm). Eighty per cent of the NF-NETs were hypointense on T1-weighted images, 82.2 % were hyperintense on T2-weighted images, and 75.6 % were hypervascular. Overall MRI accuracy showed a mean AUC of 0.86 compared to pathology. Lesions with a maximum diameter of 30 mm irregular margins, absence of a cleavage plane with the main pancreatic duct, vascular encasement, extrapancreatic spread and abdominal metastases were significantly associated with malignant NF-NETs. No correlation was found between the tumour vessel density and contrast-enhanced MR imaging pattern. CONCLUSIONS: Hyperintensity on T2-weighted images and iso-/hypervascularity occurred in 27/45 (60.0 %) of NF-NETs. MRI identifies malignant NF-NETs with a sensitivity of 93.3 % and a specificity of 76.9 % (AUC = 0.85). KEY POINTS: • Non-hyperfunctioning neuroendocrine pancreatic tumours (NF-NET) pose a difficult diagnostic challenge. • On T2-weighted MRI, 82.2 % of neuroendocrine tumours appeared hyperintense. • MR imaging showed 0.94 sensitivity and 0.77 specificity in predicting biological behaviour. • The hyper-/isointensity during dynamic MRI did not correlate with vessel density at pathology.

Autoimmune pancreatitis and non-necrotizing acute pancreatitis: computed tomography pattern.

OBJECTIVES: To retrospectively differentiate diffuse autoimmune pancreatitis from non-necrotizing acute pancreatitis at clinical onset with multi detector row computed tomography. METHODS: 36 Patients suffering from diffuse autoimmune pancreatitis (14) or non-necrotizing acute pancreatitis (22) were enrolled. Qualitative analysis included stranding, retroperitoneal fluid film, capsule-like rim enhancement and pleural effusion. In quantitative analysis pancreatic density was measured in all phases. The vascularization behaviour was assessed using the relative enhancement rate across all phases. RESULTS: Pancreatic density resulted lower in non-necrotizing acute pancreatitis compared to diffuse autoimmune pancreatitis patients in pre-contrast phase and higher in pancreatic phase. Relative enhancement rate evaluation confirmed different vascularization behaviours of the two diseases. Only non-necrotizing acute pancreatitis Patients presented peripancreatic stranding and fluid in the retromesenteric interfascial plane. CONCLUSIONS: Multi detector row computed tomography is a useful technique for differentiating diffuse autoimmune pancreatitis from non-necrotizing acute pancreatitis at clinical onset. Peripancreatic stranding and retroperitoneal fluid film, characteristic of non-necrotizing acute pancreatitis, and late-phase peripheral rim enhancement, characteristic of diffuse autoimmune pancreatitis, provide qualitative clues to the differentiation. A quantitative study of contrast enhancement patterns, considering the relative enhancement rate, can assist in the differential diagnoses of two diseases.

Autoimmune pancreatitis: pancreatic and extrapancreatic MR imaging-MR cholangiopancreatography findings at diagnosis, after steroid therapy, and at recurrence.

PURPOSE: To determine and describe the magnetic resonance (MR) imaging-MR cholangiopancreatographic pancreatic and extrapancreatic findings of autoimmune pancreatitis (AIP) and the probability, site, and MR features of recurrent AIP after steroid therapy. MATERIALS AND METHODS: This retrospective study was approved by the institutional review board, and the requirement for informed patient consent was waived. The data of 27 patients with AIP were included in the study. All patients had undergone MR imaging with MR cholangiopancreatography before and after steroid treatment and during follow-up (median follow-up period, 45 months). Image analysis included assessment of pancreatic parenchyma enlargement, signal intensity on T1- and T2-weighted MR images, contrast enhancement, and presence of bile duct and/or renal involvement. The probability of AIP recurrence was assessed by using Kaplan-Meier curves and the unadjusted Cox model. RESULTS: At the time of diagnosis, the AIP-affected pancreatic parenchyma showed diffuse enlargement in 14 (52%) of the 27 patients and segmental enlargement in 13 (48%). The pancreatic parenchyma appeared hypointense on T1-weighted images in all 27 (100%) patients, hyperintense on T2-weighted images in 25 (93%), and isointense in two (7%). During the pancreatic phase of the dynamic contrast material-enhanced study, the affected pancreatic parenchyma appeared hypointense in 25 (93%) patients and isointense in two (7%). During the portal venous and delayed phases, the images of 19 (70%) patients showed delayed enhancement. Bile duct involvement was observed in 10 (37%) patients, and renal involvement was observed in two (7%). After steroid treatment, six (22%) patients had recurrent AIP, with a median disease-free interval of 20.6 months. The sites of recurrence were the pancreas and the kidneys in three of the six patients, solely the pancreas in two patients, and the biliary ducts in one patient. CONCLUSION: MR imaging with MR cholangiopancreatography enables the diagnosis of pancreatic and extrapancreatic AIP and the assessment of changes after steroid therapy.

Italian consensus guidelines for chronic pancreatitis.

This paper gives practical guidelines for diagnosis and treatment of chronic pancreatitis. Statements have been elaborated by working teams of experts, by searching for and analysing the literature, and submitted to a consensus process by using a Delphi modified procedure. The statements report recommendations on clinical and nutritional approach, assessment of pancreatic function, treatment of exocrine pancreatic failure and of secondary diabetes, treatment of pain and prevention of painful relapses. Moreover, the role of endoscopy in approaching pancreatic pain, pancreatic stones, duct narrowing and dilation, and complications was considered. Recommendations for most appropriate use of various imaging techniques and of ultrasound endoscopy are reported. Finally, a group of recommendations are addressed to the surgical treatment, with definition of right indications, timing, most appropriate procedures and techniques in different clinical conditions and targets, and clinical and functional outcomes following surgery.

Main pancreatic duct intraductal papillary mucinous neoplasms: accuracy of MR imaging in differentiation between benign and malignant tumors compared with histopathologic analysis.

PURPOSE: To retrospectively determine the accuracy of magnetic resonance (MR) imaging combined with MR cholangiopancreatography (CP) in differentiating benign from malignant intraductal papillary mucinous neoplasms (IPMNs) involving the main pancreatic duct (MPD), with histopathologic analysis as the reference standard. MATERIALS AND METHODS: The informed consent requirement was waived for this institutional review board-approved study. A total of 51 patients with histopathologically proved IPMNs (MPD IPMN, n = 29; mixed type IPMN, n = 22), underwent MR imaging, MR CP, and surgery, with a mean interval of 2.6 months between MR examination and surgery. Qualitative image analysis included assessment of the site of MPD dilatation (head of the pancreas, body and/or tail of the pancreas, or diffuse), presence or absence of duct wall nodules, and contrast enhancement of the MPD walls. Quantitative image analysis included measurement of the maximum diameter of the MPD. A comparison of adenomas and borderline IPMNs with cancerous IPMNs was performed with the Student t test or the Mann-Whitney U test for continuous variables. RESULTS: At histopathologic analysis, 27 IPMNs were classified as carcinomas; 13, as borderline tumors; and 11, as adenomas. MPD wall nodules were observed in 16 carcinomas involving the MPD and one adenoma or borderline neoplasm (P < .00001). Duct wall enhancement was observed in 20 MPD or mixed type carcinomas and five adenomas or borderline neoplasms (P = .0001). The median maximal diameter of the MPD was 18 mm in malignant MPD or mixed type IPMNs and 11 mm in benign borderline IPMNs (P = .038). No significant difference in the overall 5-year survival rate of patients with MPD IPMNs and those with mixed type IPMNs was observed (P = .813). CONCLUSION: Duct wall nodules and enhancement of the MPD walls are signs of malignant MPD or mixed type IPMNs. SUPPLEMENTAL MATERIAL: http://radiology.rsna.org/lookup/suppl/doi:10.1148/radiol.2531080604/-/DC1.

Autoimmune pancreatitis: differences between the focal and diffuse forms in 87 patients.

OBJECTIVES: Autoimmune pancreatitis (AIP) is a particular type of chronic pancreatitis that can be classified into diffuse and focal forms. The aim of this study was to analyze clinical and instrumental features of patients suffering from the diffuse and focal forms of AIP. METHODS: AIP patients diagnosed between 1995-2008 were studied. RESULTS: A total of 87 AIP patients (54 male and 33 female patients, mean age 43.4+/-15.3 years) were studied. Focal-type AIP was diagnosed in 63% and diffuse-type in 37%. Association with autoimmune diseases was observed in 53% of cases, the most common being ulcerative colitis (30%). Serum levels of IgG4 exceeded the upper normal limits (135 mg/dl) in 66% of focal AIP and in 27% of diffuse AIP (P=0.006). All patients responded to steroids. At recurrence non-steroid immunosuppressive drugs were successfully used in six patients. Recurrences were observed in 25% of cases, and were more frequent in focal AIP (33%) than in diffuse AIP (12%) (P=0.043), in smokers than in non-smokers (41% vs. 15%; P=0.011), and in patients with pathological serum levels of IgG4 compared to those with normal serum levels (50% vs. 12%; P=0.009). In all, 23% of the patients underwent pancreatic resections. Among patients with focal AIP, recurrences were observed in 30% of operated and in 34% of not operated patients. CONCLUSIONS: Focal-type and diffuse-type AIP differ as regards clinical symptoms and signs. Recurrences occur more frequently in focal AIP than in diffuse AIP. The use of non-steroid immunosuppressants may be a therapeutic option in relapsing AIP.

Clinical and radiological outcome of patients suffering from chronic pancreatitis associated with gene mutations.

OBJECTIVES: Cystic fibrosis transmembrane conductance regulator (CFTR), cationic trypsinogen gene (PRSS1), and serine protease inhibitor kazal type 1 (SPINK1) gene mutations have been associated with chronic pancreatitis (CP). The aim of this study was to compare clinical and radiological findings in sporadic CP with (CPgm) and without (CPwt) gene mutations. METHODS: Data from patients observed between 2001 and 2006 were collected. All patients were tested for 25 CFTR gene mutations, for R122H and N29I on the PRSS1 gene, and for N34S mutation on the SPINK1 gene. RESULTS: We found 34 (17.2%) of 198 patients with CPgm, 23 (11.6%) of them on the CFTR gene, 11 (5.6%) on the SPINK1, and none on the PRSS1 gene. The age at clinical onset was younger in CPgm (36.2 +/- 17.2 years) than in CPwt (44 +/- 12.6 years; P = 0.005). There were more heavy drinkers among CPwt (33%) than among CPgm (9%; P = 0.003), and the same applied to smokers (69% vs 33%, respectively; P < 0.0001). In CPgm group, the onset of pancreatic calcifications was observed more frequently in drinkers and/or smokers. Exocrine and endocrine insufficiency occurred less frequently and later in CPgm than in CPwt patients. CONCLUSIONS: Clinical and radiological outcome differ in CPgm compared with CPwt. Alcohol, even in small quantities, and cigarette smoking influence the onset of pancreatic calcifications.

Autoimmune pancreatitis: CT patterns and their changes after steroid treatment.

PURPOSE: To retrospectively evaluate the computed tomographic (CT) patterns of autoimmune pancreatitis (AIP) and their changes after steroid therapy. MATERIALS AND METHODS: Investigational review board approval was obtained, and the informed consent requirement was waived. The medical and imaging data of 21 patients (13 men, eight women; mean age, 47.5 years; age range, 25-79 years) with histopathologically proved AIP who underwent contrast material-enhanced CT at diagnosis and after steroid treatment were included in this study. Image analysis included assessment of the (a) presence or absence and type (focal or diffuse) of pancreatic parenchyma enlargement, (b) contrast enhancement of pancreatic parenchyma, (c) size of the main pancreatic duct (MPD) within the lesion and upstream, and (d) pancreatic parenchyma thickness in the head, body, and tail of the pancreas. The same criteria were applied to follow-up CT examinations, the follow-up data were compared with pretreatment data, and a paired sample t test was applied. RESULTS: Pancreatic parenchyma showed focal enlargement in 14 (67%) patients and diffuse enlargement in seven (33%). Pancreatic parenchyma affected by AIP appeared hypoattenuating in 19 (90%) patients and isoattenuating in two (10%). During the portal venous phase, pancreatic parenchyma showed contrast material retention in 18 (86%) patients and contrast material washout in three (14%). The MPD was never visible within the lesion. After treatment, there was a reduction in the size of pancreatic parenchyma segments affected by AIP (P < .05). Fifteen (71%) of the 21 patients had a normal enhancement pattern in the pancreatic parenchyma, whereas the enhancement pattern remained hypovascular in six (29%). The MPD returned to its normal size within the lesion in all patients at follow-up CT. In one of the eight patients with focal forms of AIP, the upstream MPD remained dilated. CONCLUSION: AIP appeared as pancreatic parenchyma enlargement, with MPD stenosis within the lesion and upstream dilatation in focal forms of AIP. After steroid treatment, there was normalization of these findings.

Clinical and radiological evolution of a case of idiopathic retroperitoneal fibrosis treated with Tamoxifen.

The authors report a case of retroperitoneal fibrosis conservatively treated with Tamoxifen. The computerized tomography (TC) control at ten months showed the persistence of a slight residual of peri-aortic fibrotic tissue. This result allowed removal of the double J catheter.