European Neuroendocrine Tumor Society (ENETS) 2024 guidance paper for the management of well-differentiated small intestine neuroendocrine tumours.

Both the incidence and prevalence of well-differentiated neuroendocrine tumours from the small intestine (Si-NET) are gradually increasing. Most patients have non-functioning tumours with subtle GI symptoms and tumours are often discovered incidentally by endoscopy or at advanced disease stages by imaging depicting mesenteric lymph node and /or liver metastases while around 30% of the patients present with symptoms of the carcinoid syndrome. Adequate biochemical assessment and staging including functional imaging is crucial for treatment-related decision-making that should take place in an expert multidisciplinary team setting. Preferably, patients should be referred to specialised ENETS Centres of Excellence or centres of high expertise in the field. This guidance paper provides the current evidence and best knowledge for the management of Si-NET grade (G) 1-3 following 10 key questions of practical relevance for the diagnostic and therapeutic decision making.

Ampullary tumors: French Intergroup Clinical Practice Guidelines for diagnosis, treatments and follow-up (TNCD, SNFGE, FFCD, UNICANCER, GERCOR, SFCD, SFED, ACHBT, AFC, SFRO, RENAPE, SNFCP, AFEF, SFP, SFR).

BACKGROUND: Management of ampullary tumors (AT) is challenging because of a low level of scientific evidence. This document is a summary of the French intergroup guidelines regarding the management of AT, either adenoma (AA) or carcinoma (AC), published in July 2023, available on the website of the French Society of Gastroenterology (SNFGE) (www.tncd.org). METHODS: A collaborative work was conducted under the auspices of French medical, endoscopic, oncological and surgical societies involved in the management of AT. Recommendations are based on recent literature review and expert opinions and graded in three categories (A, B, C), according to quality of evidence. RESULTS: Accurate diagnosis of AT requires at least duodenoscopy and EUS. All patients should be discussed in multidisciplinary tumor board before treatment. Surveillance may only be proposed for small AA in familial adenomatous polyposis. For AA, endoscopic papillectomy is the preferred option only if R0 resection can be achieved. When not possible, surgical papillectomy should be considered. For AC beyond pT1a N0, pancreaticoduodenectomy is the procedure of choice. Adjuvant monochemotherapy (gemcitabine, 5FU) may be proposed. For aggressive tumors (pT3/T4, pN+, R1, poorly differentiated AC, pancreatobiliary differentiation) with high risk of recurrence, 6 months polychemotherapy (CAPOX/FOLFOX for the intestinal subtype and mFOLFIRINOX for the pancreatobiliary or the mixed subtype) may be a valid alternative. Clinical and radiological follow up is recommended for 5 years. CONCLUSIONS: These guidelines help to homogenize and highlight unmet needs in the management of AA and AC. Each individual case should be discussed by a multidisciplinary team.

Appropriate use of morphological imaging for assessing treatment response and disease progression of neuroendocrine tumors.

Neuroendocrine tumors (NETs) are relatively rare neoplasms displaying heterogeneous clinical behavior, ranging from indolent to aggressive forms. Patients diagnosed with NETs usually receive a varied array of treatments, including somatostatin analogs, locoregional treatments (ablation, intra-arterial therapy), cytotoxic chemotherapy, peptide receptor radionuclide therapy (PRRT), and targeted therapies. To maximize therapeutic efficacy while limiting toxicity (both physical and economic), there is a need for accurate and reliable tools to monitor disease evolution and progression and to assess the effectiveness of these treatments. Imaging morphological methods, primarily relying on computed tomography (CT) and magnetic resonance imaging (MRI), are indispensable modalities for the initial evaluation and continuous monitoring of patients with NETs, therefore playing a pivotal role in gauging the response to treatment. The primary goal of assessing tumor response is to anticipate and weigh the benefits of treatments, especially in terms of survival gain. The World Health Organization took the pioneering step of introducing assessment criteria based on cross-sectional imaging. This initial proposal standardized the measurement of lesion sizes, laying the groundwork for subsequent criteria. The Response Evaluation Criteria in Solid Tumors (RECIST) subsequently refined and enhanced these standards, swiftly gaining acceptance within the oncology community. New treatments were progressively introduced, targeting specific features of NETs (such as tumor vascularization or expression of specific receptors), and achieving significant qualitative changes within tumors, although associated with minimal or paradoxical effects on tumor size. Several alternative criteria, adapted from those used in other cancer types and focusing on tumor viability, the slow growth of NETs, or refining the existing size-based RECIST criteria, have been proposed in NETs. This review article aims to describe and discuss the optimal utilization of CT and MRI for assessing the response of NETs to treatment; it provides a comprehensive overview of established and emerging criteria for evaluating tumor response, along with comparative analyses. Molecular imaging will not be addressed here and is covered in a dedicated article within this special issue.

European Neuroendocrine Tumor Society 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromes.

This ENETS guidance paper aims to provide practical advice to clinicians for the diagnosis, treatment and follow-up of functioning syndromes in pancreatic neuroendocrine tumours (NET). A NET-associated functioning syndrome is defined by the presence of a clinical syndrome combined with biochemical evidence of inappropriately elevated hormonal levels. Different hormonal syndromes can be encountered in pancreatic NET patients, including insulinoma, gastrinoma as well as the rare glucagonoma, VIPoma, ACTHoma, PTHrPoma, carcinoid syndrome, calcitoninoma, GHRHoma and somatostatinoma. The recommendations provided in this paper focus on the biochemical, genetic and imaging work-up as well as therapeutic management of the individual hormonal syndromes in well-differentiated, grade 1-3, functioning NET with the primary tumour originating in the pancreas, and for specific subtypes also in the duodenum.

Automatic Detection of Pancreatic Lesions and Main Pancreatic Duct Dilatation on Portal Venous CT Scans Using Deep Learning.

OBJECTIVES: This study proposes and evaluates a deep learning method to detect pancreatic neoplasms and to identify main pancreatic duct (MPD) dilatation on portal venous computed tomography scans. MATERIALS AND METHODS: A total of 2890 portal venous computed tomography scans from 9 institutions were acquired, among which 2185 had a pancreatic neoplasm and 705 were healthy controls. Each scan was reviewed by one in a group of 9 radiologists. Physicians contoured the pancreas, pancreatic lesions if present, and the MPD if visible. They also assessed tumor type and MPD dilatation. Data were split into a training and independent testing set of 2134 and 756 cases, respectively.A method to detect pancreatic lesions and MPD dilatation was built in 3 steps. First, a segmentation network was trained in a 5-fold cross-validation manner. Second, outputs of this network were postprocessed to extract imaging features: a normalized lesion risk, the predicted lesion diameter, and the MPD diameter in the head, body, and tail of the pancreas. Third, 2 logistic regression models were calibrated to predict lesion presence and MPD dilatation, respectively. Performance was assessed on the independent test cohort using receiver operating characteristic analysis. The method was also evaluated on subgroups defined based on lesion types and characteristics. RESULTS: The area under the curve of the model detecting lesion presence in a patient was 0.98 (95% confidence interval [CI], 0.97-0.99). A sensitivity of 0.94 (469 of 493; 95% CI, 0.92-0.97) was reported. Similar values were obtained in patients with small (less than 2 cm) and isodense lesions with a sensitivity of 0.94 (115 of 123; 95% CI, 0.87-0.98) and 0.95 (53 of 56, 95% CI, 0.87-1.0), respectively. The model sensitivity was also comparable across lesion types with values of 0.94 (95% CI, 0.91-0.97), 1.0 (95% CI, 0.98-1.0), 0.96 (95% CI, 0.97-1.0) for pancreatic ductal adenocarcinoma, neuroendocrine tumor, and intraductal papillary neoplasm, respectively. Regarding MPD dilatation detection, the model had an area under the curve of 0.97 (95% CI, 0.96-0.98). CONCLUSIONS: The proposed approach showed high quantitative performance to identify patients with pancreatic neoplasms and to detect MPD dilatation on an independent test cohort. Performance was robust across subgroups of patients with different lesion characteristics and types. Results confirmed the interest to combine a direct lesion detection approach with secondary features such as the MPD diameter, thus indicating a promising avenue for the detection of pancreatic cancer at early stages.

Proposal of early CT morphological criteria for response of liver metastases to systemic treatments in gastroenteropancreatic neuroendocrine tumors: Alternatives to RECIST.

RECIST 1.1 criteria are commonly used with computed tomography (CT) to evaluate the efficacy of systemic treatments in patients with neuroendocrine tumors (NETs) and liver metastases (LMs), but their relevance is questioned in this setting. We aimed to explore alternative criteria using different numbers of measured LMs and thresholds of size and density variation. We retrospectively studied patients with advanced pancreatic or small intestine NETs with LMs, treated with systemic treatment in the first-and/or second-line, without early progression, in 14 European expert centers. We compared time to treatment failure (TTF) between responders and non-responders according to various criteria defined by 0%, 10%, 20% or 30% decrease in the sum of LM size, and/or by 10%, 15% or 20% decrease in LM density, measured on two, three or five LMs, on baseline (≤1 month before treatment initiation) and first revaluation (≤6 months) contrast-enhanced CT scans. Multivariable Cox proportional hazard models were performed to adjust the association between response criteria and TTF on prognostic factors. We included 129 systemic treatments (long-acting somatostatin analogs 41.9%, chemotherapy 26.4%, targeted therapies 31.8%), administered as first-line (53.5%) or second-line therapies (46.5%) in 91 patients. A decrease ≥10% in the size of three LMs was the response criterion that best predicted prolonged TTF, with significance at multivariable analysis (HR 1.90; 95% CI: 1.06-3.40; p = .03). Conversely, response defined by RECIST 1.1 did not predict prolonged TTF (p = .91), and neither did criteria based on changes in LM density. A ≥10% decrease in size of three LMs could be a more clinically relevant criterion than the current 30% threshold utilized by RECIST 1.1 for the evaluation of treatment efficacy in patients with advanced NETs. Its implementation in clinical trials is mandatory for prospective validation. Criteria based on changes in LM density were not predictive of treatment efficacy. CLINICAL TRIAL REGISTRATION: Registered at CNIL-CERB, Assistance publique hopitaux de Paris as "E-NETNET-L-E-CT" July 2018. No number was assigned. Approved by the Medical Ethics Review Board of University Medical Center Groningen.

Dilatation of the main pancreatic duct of unknown origin: causes and risk factors of pre-malignancy or malignancy.

INTRODUCTION: A dilatation of the main pancreatic duct (MPD) is mainly due to obstructive causes (pancreatic tumor, chronic pancreatitis) or intraductal papillary mucinous neoplasm (IPMN). This study aims to assess the risk of pre-malignancy or malignancy in case of MPD dilatation with no visible mass nor obstructive calcification on computed tomography scan (CT-scan) in a population operated for it. PATIENTS AND METHODS: All patients operated on from November 2015 to December 2019 in our center for a significant dilatation of the MPD without visible obstructive cause on CT-scan were included. Preoperative work-up included at least CT-scan, magnetic resonance imaging (MRI), and endoscopic ultrasonography (EUS). Primary endpoint was the final pathological diagnosis. Secondary endpoints were predictive factors of malignancy. RESULTS: 101 patients were included, mean age 68 years-old. Final pathological data were pancreatic adenocarcinoma (n = 2), IPMN with high-grade dysplasia (n = 37), high-grade Pancreatic Intraepithelial Neoplasia (PanIN) (n = 2) (total of pre-malignant or malignant lesions: n = 41), neuroendocrine tumor (n = 6), IPMN with low-grade dysplasia (n = 45), low-grade PanIN (n = 5), chronic pancreatitis (n = 3), and benign stenosis (n = 1). On preoperative explorations, the median diameter of MPD was 7 mm [3-35]. MRI and/or EUS showed intraductal material, nodule, or cyst in 22, 32, and 52 patients, respectively; 22 patients without nodule visible on MRI or EUS had still a pre-malignant or malignant lesion. In multivariate analysis, predictive factors for pre-malignancy or malignancy were symptoms before surgery (p = 0.01), MPD dilatation without downstream stenosis (p = 0.046), and the presence of nodule (p = 0.009). CONCLUSION: A dilatation of the MPD without detectable mass or obstructive calcification on CT-scan was associated with a pre-malignant or malignant lesion in 41 patients. Symptoms before surgery, MPD dilatation without duct narrowing, and the presence of nodules on MRI/EUS were associated with the risk of  pre-malignancy or malignancy.

Volumetric Enhancing Tumor Burden at CT to Predict Survival Outcomes in Patients with Neuroendocrine Liver Metastases after Intra-arterial Treatment.

Purpose To investigate whether liver enhancing tumor burden (LETB) assessed at contrast-enhanced CT indicates early response and helps predict survival outcomes in patients with multifocal neuroendocrine liver metastases (NELM) after intra-arterial treatment. Materials and Methods This retrospective study included patients with NELM who underwent intra-arterial treatment with transarterial embolization (TAE) or chemoembolization (TACE) between April 2006 and December 2018. Tumor response in treated NELM was evaluated by using the Response Evaluation Criteria in Solid Tumors (RECIST) and modified RECIST (mRECIST). LETB was measured as attenuation 2 SDs greater than that of a region of interest in the nontumoral liver parenchyma. Overall survival (OS); time to unTA(C)Eable progression, defined as the time from the initial treatment until the time when intra-arterial treatments were considered technically unfeasible, either not recommended by the multidisciplinary tumor board or until death; and hepatic and whole-body progression-free survival (PFS) were evaluated using multivariable Cox proportional hazards analyses, the Kaplan-Meier method, and log-rank test. Results The study included 119 patients (mean age, 60 years ± 11 [SD]; 61 men) who underwent 161 treatments. A median LETB change of -25.8% best discriminated OS (83 months in responders vs 51 months in nonresponders; P = .02) and whole-body PFS (18 vs 8 months, respectively; P < .001). A -10% LETB change best discriminated time to unTA(C)Eable progression (32 months in responders vs 12 months in nonresponders; P < .001) and hepatic PFS (18 vs 8 months, respectively; P < .001). LETB change remained independently associated with improved OS (hazard ratio [HR], 0.56), time to unTA(C)Eable progression (HR, 0.44), hepatic PFS (HR, 0.42), and whole-body PFS (HR, 0.47) on multivariable analysis. Neither RECIST nor mRECIST helped predict patient outcome. Conclusion Response according to LETB change helped predict survival outcomes in patients with NELM after intra-arterial treatments, with better discrimination than RECIST and mRECIST. Keywords: CT, Chemoembolization, Embolization, Abdomen/GI, Liver Supplemental material is available for this article. © RSNA, 2023.

ENETS standardized (synoptic) reporting for radiological imaging in neuroendocrine tumours.

This expert consensus document represents an initiative by the European Neuroendocrine Tumor Society (ENETS) to provide guidance for synoptic reporting of radiological examinations critical to the diagnosis, grading, staging and treatment of neuroendocrine neoplasms (NENs). Template drafts for initial tumor staging and follow-up by computed tomography (CT) and magnetic resonance imaging (MRI) were established, based on existing institutional and organisational reporting templates relevant for NEN imaging, and applying the RadLex lexicon of radiological information (Radiological Society of North America), for consistency regarding the radiological terms. During the ENETS Scientific Advisory Board meeting 2018, the template drafts were subject to iterative interdisciplinary discussions among experts in imaging, surgery, gastroenterology, oncology and pathology. Members of the imaging group stated a strong preference for a combination of limited and standardised options by way of drop-down menus. Separate templates were produced for the initial work-up and for follow-up, respectively. To provide a detailed description of the radiological findings of the primary tumor and its local extension and spread, different templates were developed for bronchial, pancreatic and gastrointestinal NENs for CT and MRI, respectively. Each template was structured in 10 sections: clinical details, comparative imaging modality, acquisition technique, primary tumor findings, regional lymph node metastases, distant metastases, TNM classification, reference lesions according to RECIST 1.1, additional findings and conclusion. Two templates were developed for follow-up, for CT and MRI, respectively, and were specifically focused on assessment of therapy response. These included a qualitative response assessment, such as decrease of vascularisation and presence of necrosis, and a quantitative assessment according to RECIST 1.1 and the modified RECIST (mRECIST) for assessing tumor response following transarterial chemoembolisation.

MRI is useful to suggest and exclude malignancy in mucinous cystic neoplasms of the pancreas.

OBJECTIVES: To evaluate the value of MRI in differentiating benign (b-MCN) and malignant (m-MCN) MCN. European guidelines suggest that certain mucinous cystic neoplasms (MCN) of the pancreas can be conservatively managed. METHODS: A retrospective single-center study of consecutive patients with resected MCN. MRIs were independently reviewed by two readers blinded to the pathological results. The authors compared b-MCN (i.e., mucinous-cystadenoma comprising high-grade dysplasia (HGD)) and m-MCN (i.e., cystadenocarcinoma). RESULTS: Sixty-three patients (62 women [98%]) with 63 MCN (6 m-MCN, 2 HGD) were included. m-MCN tumors had a tendency to be larger than b-MCN (median 86 [25-103] vs. 45 [17-130] mm, p = .055). The combination of signal heterogeneity on T2-weighted imaging, wall thickness ≥ 5 mm, the presence of mural nodules ≥ 9 mm, and enhancing septa had an area under the ROC curve of 0.97 (95% CI 0.91-1.00) for the diagnosis of m-MCN. A total of 24 (37%), 20 (32%), 10 (16%), 5 (8%), and 4 (6%) out of 63 MCNs showed 0, 1, 2, 3, and 4 of these features, respectively. The corresponding rate of m-MCN was 0%, 0%, 10%, 20%, and 100%, respectively, with a good-to-excellent inter-reader agreement. Patterns with a high NPV for m-MCN included an absence of enhancing septa or walls (NPV 97% and 100%, respectively), wall thickness < 3 mm (NPV 100%), and no mural nodules (NPV 100%). CONCLUSIONS: A combination of 4 imaging features suggests malignant MCN on MRI. On the other hand, visualization of a thin non-enhancing wall with no mural nodules suggests benign MCN. KEY POINTS: • A heterogenous signal on T2-weighted MRI, a ≥ 5-mm-thick wall, mural nodules ≥ 9 mm, and/or enhancing septa suggest malignant MCNs. • A thin non-enhancing wall with no mural nodules suggests benign MCNs. • MRI should be performed in the pre-therapeutic evaluation of MCN to help determine the therapeutic strategy in these patients.

Diffuse Versus Localized Caroli Disease: A Comparative MRCP Study.

OBJECTIVE. The purpose of this multicenter retrospective study was to assess the MRCP features of Caroli disease (CD). MATERIALS AND METHODS. Sixty-six patients were identified from 2000 to 2019. The inclusion criteria were diagnosis of diffuse or localized CD mentioned in an imaging report, presence of intrahepatic bile duct (IHBD) dilatation, and having undergone an MRCP examination. The exclusion criteria included presence of obstructive proximal biliary stricture and having undergone hepatobiliary surgery other than cholecystectomy. Histopathology records were available for 53 of the 66 (80%) patients. Diffuse and localized diseases were compared by chi-square and t tests and Kaplan-Meier model. RESULTS. Forty-five patients had diffuse bilobar CD ((five pediatric patients [three girls and two boys] with a mean [± SD] age of 8 ± 5 years [range, 1-15 years] and 40 adult patients [26 men and 14 women] with a mean age of 35 ± 11 years [range, 20-62 years]) and 21 patients had localized disease (12 men and 9 women; mean age, 54 ± 14 years). Congenital hepatic fibrosis was found only in patients with diffuse CD (35/45 [78%]), as was a "central dot" sign (15/35 [43%]). IHBD dilatation with both saccular and fusiform features was found in 43 (96%) and the peripheral "funnel-shaped" sign in 41 (91%) of the 45 patients with diffuse CD but in none of the patients with localized disease (p < .001). Intrahepatic biliary calculi were found in all patients with localized disease but in only 16 of the 45 (36%) patients with diffuse CD (p < .001). Left liver atrophy was found in 18 of the 21 (86%) patients with localized disease and in none of the patients with diffuse CD (p < .001). The overall survival rate among patients with diffuse CD was significantly lower than that among patients with localized disease (p = .03). CONCLUSION. Diffuse IHBD dilatation with both saccular and fusiform features associated with the peripheral funnel-shaped sign can be used for the diagnosis of CD on MRCP. Localized IHBD dilatation seems to be mainly related to primary intrahepatic lithiasis.

Are recist criteria adequate in assessing the response to therapy in metastatic NEN?

Response to therapy criteria, known as RECIST (Response Evaluation Criteria in Solid Tumours), are widely used to evaluate neuroendocrine tumours (NET) metastatic to the liver, under treatment. RECIST criteria does not take in account many various distinct features such as tumour growth, secretory capacity and anatomical localisation with wide variation in clinical and biological presentation of different NETs. Key features of RECIST includes definitions of the minimal size of measurable lesions, instructions on how many lesions to measure and follow, and the use of unidimensional, rather than bidimensional, measures for overall evaluation of tumour burden. These measures are currently done with computed tomography (CT) or Magnetic Resonance Imaging (MRI). RECIST criteria are accurate in assessing tumour progression but sometimes inaccurate in assessing tumour response after locoregional therapy or under molecular targeted therapy, tumour vessels being part of the target of such treatments. There is poor correlation between a so called tumour necrosis and conventional methods of response assessment, which poses questions of how best to quantify efficacy of these targeted therapies. Variations in tumour density with computed tomography (CT) could theoretically be associated with tumour necrosis. This hypothesis has been studied proposing alternative CT criteria of response evaluation in metastatic digestive NET treated with targeted therapy. If preliminary results upon the poor relationship between density measured with CT (derived from CHOI criteria) evolution curves at CT and PFS are confirmed by further studies, showing that the correlation between density changing and response to non-targeted treatment is weak, the use of contrast injection, will probably be not mandatory to enable appropriate evaluation.

French reconnection: A conservative pancreato-enteric reconnection for disconnected pancreatic duct syndrome.

BACKGROUND/OBJECTIVES: Disconnectedpancreatic duct syndrome (DPDS), a severe complication of acute necrotizing pancreatitis (ANP), may require surgery, usually by distal splenopancreatectomy, thus increasing the risk of diabetes. We describe a new technique reconnecting the distal pancreas to the digestive tract. METHODS: This technique was proposed after failure of non-surgical treatment and at least 3 months after the onset of ANP in non-diabetic or non-insulin dependent diabetic patients with a distal pancreas of at least 5 cm. The ruptured zone was identified and the distal side was anastomosed to the stomach or the jejunum. RESULTS: From 2013 to June 2019, 36 patients (median age = 49 years) with DPDS underwent a "French reconnection" procedure, indicated for chronic pain/recurrent pancreatitis (n = 35; 97%), persistent pancreatic fistula (n = 33; 91%), or digestive compression/fistulisation (n = 9; 25%). Median preoperative weight loss was 10 kg (4-27), the median number of hospitalisations per patient was 5(1-8) and 24(67%) patients had received endoscopic/percutaneous treatment. Surgery was performed in median 279(90-2000) days after ANP, laparoscopically in 9(25%) patients. The remnant pancreas (median length = 70 mm; range = 50-130) was anastomosed to the stomach (n = 30) or the jejunum (n = 6). There were 13(36%) postoperative grade B/C pancreatic fistulas and 3(10%) bleedings including one death (mortality = 3%). The median hospital stay was 18 (7-121) days. After a median follow-up of 24 (4-53) months, all pancreatic fistulas had healed and the clinical success rate was 91%. Median BMI increased from 22 to 25 kg/m2. In patients with normal pancreatic function, postoperative de novo endocrine and severe exocrine insufficiencies were observed in 4/27 (15%) and 7/22 (32%), respectively. CONCLUSIONS: The "French reconnection" procedure, as an alternative to distal splenopancreatectomy for the treatment of DPDS, provides good control of symptoms and decreases the risk of pancreatic insufficiency.

Recurrent acute pancreatitis caused by combined gastric and pancreatic duplications: value of preoperative CT and MRCP and conservative surgery.

We present a series of patients with recurrent acute pancreatitis caused by a duplicated pancreatic head connected to a gastric duplication and successfully treated by conservative surgery. This retrospective study included consecutive adult patients referred to our institution for recurrent acute pancreatitis. All patients underwent a preoperative non-invasive imaging examination including contrast-enhanced computed tomography and magnetic resonance cholangiopancreatography (MRCP). The final diagnosis of this developmental anomaly was based on surgical and pathological examinations. The four patients in this study had the same typical imaging pattern including a duplicated duct. There was no recurrent acute pancreatitis after surgical treatment, which involved atypical resection of the duplicated pancreatic head and segmental gastric resection, without a Whipple procedure. The discovery of an accessory pancreatic head with a duct terminating in a cyst identified on MRCP in a patient with recurrent acute pancreatitis could suggest this rare and surgically treatable cause of acute pancreatitis.

European Guideline on IgG4-related digestive disease - UEG and SGF evidence-based recommendations.

The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6-0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2-4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added.

Correction to: Prediction of pancreatic neuroendocrine tumour grade with MR imaging features: added value of diffusion-weighted imaging.

The original version of this article, published on 19 August 2016, unfortunately contained a mistake.