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Objective Myelofibrosis is a rare chronic myelolymphoproliferative disease and is associated with increased risk of venous thromboembolism. The objective of this study is to retrospectively evaluate patients with primary myelofibrosis who underwent abdominal US, MDCT and MRI, in order to identify the development of portal thrombosis and its correlation with portal-biliary cavernoma. Methods We evaluated 125 patients with initial diagnosis of primary myelofibrosis and nonspecific abdominal pain who had undergone US with color Doppler. In 13 patients (8 men, 5 females; age: 45–85), US detected portal thrombosis with associated portal-biliary cavernoma. All patients subsequently underwent contrast-enhanced MDCT and MRI and 4 patients MR-cholangiography. The correlation between primary myelofibrosis and portal thrombosis and cavernoma respectively was calculated using χ2 test. Results About 10% of patients with primary myelofibrosis preliminary evaluated with US had partial (8 pts) or complete (5 pts) portal thrombosis associated with portal-biliary cavernoma with a χ2 = 0. In all patients, US detected a concentric thickening of main bile duct (MBD) wall (mean value: 7 mm); color Doppler always showed dilated venous vessels within the thickened wall of the biliary tract. Contrast-enhanced CT and MRI confirmed thickening of MBD walls with their progressive enhancement and allowed better assessment of the extent of the portal system thrombosis. MR-cholangiography showed a thin appearance of the MBD lumen with evidence of ab extrinsic compression. Conclusions The evidence of portal thrombosis and portal-biliary cavernoma in 10% of the patients with primary myelofibrosis indicates a close correlation between the two diseases. In the detection of portal thrombosis and portal-biliary cavernoma, US with color Doppler is the most reliable and economical diagnostic technique while contrast-enhanced MDCT and MRI allow better assessment of the extent of the portal vein thrombosis and of the complications of myelofibrosis.

Introduction: The efficacy of 177Lu-Dotatate was shown in the NETTER-1 trial, an international, open-label, multicentre phase III clinical trial that evaluated the safety and efficacy of 177Lu-Dotatate in patients with well-differentiated, advanced midgut neuroendocrine tumours (NETs) with evidence of disease progression. Recently, retreatment with peptide receptor radionuclide therapy (PRRT) has been proposed as a valid therapeutic option in patients without other effective options who had responded to initial PRRT; however, data on this therapeutic option are still inadequate. Case Report: In this report, we present the case of a patient who achieved a delayed complete radiological response after initial 177Lu-Dotatate treatment and who had a complete tumour response with PRRT retreatment 5 years later. Conclusions: This case report shows that, although rare, a complete, prolonged tumour response may occur in patients with advanced small-bowel NETs receiving PRRT. Retreatment with PRRT may be a valid option in cases of subsequent disease recurrence.

Pediatric intestinal pseudoobstruction (PIPO) is the "tip of the iceberg" of the most severe gut motility disorders. In patients with PIPO, the impairment of gastrointestinal propulsive patterns is such as to result in progressive obstructive symptoms without evidence of mechanical causes. PIPO is an important cause of intestinal failure and affects growth and pubertal development. Bowel loop and abdominal distension represent one of the main features of intestinal pseudo-obstruction syndromes, hence intestinal decompression is a mainstay in the management of PIPO. So far, pharmacologic, endoscopic, and surgical treatments failed to achieve long-term relief of bowel distension and related symptoms, including pain. Recent data, however, indicated that percutaneous endoscopic gastrojejunostomy (PEG-J) might be a minimally invasive approach for intestinal decompression, thereby improving abdominal symptoms and nutritional status in adult patients with chronic intestinal pseudo-obstruction. Based on these promising results, we treated for the first time a 12-y-old patient affected by PIPO refractory to any therapeutic options to obtain intestinal decompression by PEG-J. We showed that PEG-J yielded sustained small bowel decompression in the reported PIPO patient with considerable improvement of both abdominal symptoms and nutritional status. The positive outcome of the present case provides a basis to test the actual efficacy PEG-J versus other therapeutic approaches to intestinal decompression in patients with PIPO.

Sarcopenia has been associated with poor outcomes in patients with cirrhosis and solid tumours.Analyse the influence of sarcopenia on survival and treatment duration in patients with advanced hepatocellular carcinoma (HCC) treated with sorafenib.We conducted a multicentre, retrospective study on 96 patients with advanced HCC treated with sorafenib, all with available abdominal computed tomography (CT) scan within 30 days from treatment start. Anthropometric, laboratory, treatment and follow-up data were collected. Sarcopenia was defined by reduced skeletal muscle index calculated from an L3 section CT image.Sarcopenia was present in 49% of patients. Patients were divided into two groups according to sarcopenia: age was significantly higher in the sarcopenic group (SG) (66 years (31-87) versus 72 years (30-84),Sarcopenia is present in almost half of patients with advanced HCC, and is associated with reduced survival and reduced duration of oral chemotherapy.

Background Currently, there are no data available on the best choice of treatment in heavily pretreated patients with advanced breast cancer. However, the combination of oral vinorelbine and capecitabine has been demonstrated to be effective and safe in patients with advanced breast cancer pretreated with anthracycline. Furthermore, some studies assessed the activity of dasatinib, an oral tyrosine kinase inhibitor that inhibits five oncogenic tyrosine kinase families, alone or in combination with different chemotherapy in patients affected with advanced breast cancer. Case presentation A patient with metastatic breast cancer, hormone receptor positive and human epidermal grow factor receptor 2 negative, pretreated with epirubicine, taxanes and nab-paclitaxel, was submitted to third line chemotherapy with vinorelbine 60 mg/m2 on day 1, 8 plus capecitabine 1000 mg/m2 twice daily from day 1 to day 14 every 21 days. The patient was taking also dasatinib 100 mg once daily for chronic myeloid leukemia. The treatment was well tolerated and, after 15 months, computed tomography scan showed a complete response of liver metastases and bone stable disease. After another 28 months, a 18-fluorodeoxyglucose positron emission tomography scan showed a metabolic response of bone metastases without other site of disease. Conclusions This is the first case in literature about activity of dasatinib in combination with a chemotherapy schedule of oral vinorelbine and capecitabine in advanced breast cancer. This treatment showed both good tolerability and great activity with a long progression free survival of 54 months.

Introduction: Surveillance programs on high-risk individuals (HRIs) can detect pre-malignant lesions or early pancreatic cancer (PC). We report the results of the first screening round of the Italian multicenter program supported by the Italian Association for the study of the Pancreas (AISP). Methods: The multicenter surveillance program included asymptomatic HRIs with familial (FPC) or genetic frailty (GS: BRCA1/2, p16/CDKN2A, STK11/LKB1or PRSS1, mutated genes) predisposition to PC. The surveillance program included at least an annual magnetic resonance cholangio pancreatography (MRCP). Endoscopic ultrasound (EUS) was proposed to patients who refused or could not be submitted to MRCP. Results: One-hundreds eighty-seven HRIs underwent a first-round screening examination with MRCP (174; 93.1%) or EUS (13; 6.9%) from September 2015 to March 2018.The mean age was 51 years (range 21–80).One-hundreds sixty-five (88.2%) FPC and 22 (11.8%) GF HRIs were included. MRCP detected 28 (14.9%) presumed branch-duct intraductal papillary mucinous neoplasms (IPMN), 1 invasive carcinoma/IPMN and one low-grade mixed-type IPMN, respectively. EUS detected 4 PC (2.1%): 1 was resected, 1 was found locally advanced intraoperatively, and 2 were metastatic. Age > 50 (OR 3.3, 95%CI 1.4–8), smoking habit (OR 2.8, 95%CI 1.1–7.5), and having > 2 relatives with PC (OR 2.7, 95%CI 1.1–6.4) were independently associated with detection of pre-malignant and malignant lesions. The diagnostic yield for MRCP/EUS was 24% for cystic lesions. The overall rate of surgery was 2.6% with nil mortality. Discussion: The rate of malignancies found in this cohort was high (2.6%). According to the International Cancer of the Pancreas Screening Consortium the screening goal achievement was high (1%).

Simple Summary This Part II is an overview of the main applications of Radiomics in oncologic imaging with a focus on diagnosis, prognosis prediction and assessment of response to therapy in thoracic, genito-urinary, breast, neurologic, hematologic and musculoskeletal oncology. In this part II we describe the radiomic applications, limitations and future perspectives for each pre-eminent tumor. In the future, Radiomics could have a pivotal role in management of cancer patients as an imaging tool to support clinicians in decision making process. However, further investigations need to obtain some stable results and to standardize radiomic analysis (i.e., image acquisitions, segmentation and model building) in clinical routine. Abstract Radiomics has the potential to play a pivotal role in oncological translational imaging, particularly in cancer detection, prognosis prediction and response to therapy evaluation. To date, several studies established Radiomics as a useful tool in oncologic imaging, able to support clinicians in practicing evidence-based medicine, uniquely tailored to each patient and tumor. Mineable data, extracted from medical images could be combined with clinical and survival parameters to develop models useful for the clinicians in cancer patients’ assessment. As such, adding Radiomics to traditional subjective imaging may provide a quantitative and extensive cancer evaluation reflecting histologic architecture. In this Part II, we present an overview of radiomic applications in thoracic, genito-urinary, breast, neurological, hematologic and musculoskeletal oncologic applications.

Coronavirus disease 2019 (COVID-19) is a respiratory syndrome caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) first described in Wuhan, Hubei Province, China in the last months of 2019 and then declared as a pandemic. Typical symptoms are represented by fever, cough, dyspnea and fatigue, but SARS-CoV-2 infection can also cause gastrointestinal symptoms (vomiting, diarrhoea, abdominal pain, loss of appetite) or be totally asymptomatic. As reported in literature, many patients with COVID-19 pneumonia had a secondary abdominal involvement (bowel, pancreas, gallbladder, spleen, liver, kidneys), confirmed by laboratory tests and also by radiological features. Usually the diagnosis of COVID-19 is suspected and then confirmed by real-time reverse-transcription-polymerase chain reaction (RT-PCR), after the examination of the lung bases of patients, admitted to the emergency department with abdominal symptoms and signs, who underwent abdominal-CT. The aim of this review is to describe the typical and atypical abdominal imaging findings in patients with SARS-CoV-2 infection reported since now in literature.

Purpose The aim is to determine the accuracy of magnetic resonance enterography (MRE) in evaluating Crohn's disease (CD) activity. Materials and methods Seventy-seven patients with CD underwent MRE. The primary analysis was to determine associations between MRE findings, Harvey–Bradshaw Index, and C-reactive protein (CRP), then we have created a new MRE score that it was also correlated with clinical and laboratory data. Results MRE score for CD significantly correlates with CRP ( P =.003). Significant associations were found between degree of contrast enhancement and CRP ( P =.002) and between comb sign and CRP ( P =.001). Conclusions These results make MRE an important instrument for evaluation of CD activity.

Background Sarcopenia is a complication and independent risk factor for mortality in patients with liver cirrhosis. Aim To assess the prevalence and influence of sarcopenia on overall survival in a cohort of cirrhotic patients with hepatocellular carcinoma managed in a tertiary center. Material and Methods Abdominal computed tomography of 92 consecutive hepatocellular carcinoma cirrhotic patients, enrolled and followed from 2004 to 2014, were retrospectively studied with a software analyzing the cross-sectional areas of muscles at third lumbar vertebra level. Data was normalized for height, skeletal muscle index (SMI) calculated and presence of Sarcopenia measured. Sarcopenia was defined by SMI ≤ 41 cm2/m2 for women and ≤ 53 cm2/m2 for men with body mass index (BMI) ≥ 25, and ≤ 43 cm2/m2 for men and women with BMI Results Median age at diagnosis was 71.9 years (30.7-86.4) and BMI 24.7 (17.5-36.7), comparable in women 23.1, (17.5-36.7) and men 24.7 (18.4-36.7). A class of CHILD score and BCLC A prevailed (55.4% and 41.3%, respectively); metastatic disease was found in 12% of cases. Sarcopenia was present in 40.2% of cases, mostly in females (62.9%; p = 0.005). Mean overall survival was reduced in sarcopenic patients, 66 (95% CI 47 to 84) vs. 123 (95% CI 98 to 150) weeks (p = 0.001). At multivariate analysis, sarcopenia was a predictor of reduced overall survival, independent of age (p = 0.0027). Conclusions This retrospective study shows high prevalence of sarcopenia among cirrhotic patients with hepatocellular carcinoma. Presence of sarcopenia was identified as independent predictor of reduced overall survival. As easily measurable by CT, sarcopenia should be determined for prognostic purposes in this patient population.

Background Although re-assessment of proliferative activity by K67 evaluation during the course of neuroendocrine neoplasms (NENs) is recommended in selected patients, its impact on patients’ management is not clear due to the lack of data supporting this practice. Aim To investigate Ki67 change at time of progressive disease (PD) in entero-pancreatic NENs (EP-NENs). Patients and methods Retrospective analysis of sporadic EP-NENs which received histological re-assessment after PD once radiologically documented. Results Forty-three patients were evaluated, including 24 pancreatic NENs (PNENs), and 19 small intestine NENs (SI-NENs). At time of initial histological evaluation, 19 patients had grade 1 (G1) NETs (44.2%), and 24 grade 2 (G2) NETs (55.8%), overall median Ki67 being 3% (range 1%-20%). At time of PD, 13 patients had G1 NETs (30.2%), 26 G2 NETs (60.5%), and 4 had grade 3 (G3) NECs (9.3%), thus resulting in a significant median Ki67 increase (8%, range 1%-70%; p = 0.0006), and a G upgrading in 12 patients (27.9%). A statistically significant Ki67 increase and G grading change at time of PD was observed in PNENs (p = 0.0005 and p = 0.028, respectively). Conversely, no statistically significant change occurred in non-PNENs. Conclusions In PNENs with documented PD, Ki67 increase occurs in a significant proportion of patients, providing useful information necessary to choose appropriate therapeutic options.

Objectives Transabdominal ultrasonography (US) usually reveals diagnosis of biliary acute pancreatitis (AP). Guidelines suggest repeating US in AP patients without cause at first examination. This approach has been poorly investigated, as well as the accuracy of repeated US as compared with that of magnetic resonance cholangiopancreatography. This study aims at evaluating the diagnostic accuracy of repeated US for biliary AP. Methods The accuracy of each test for diagnosis of biliary AP was evaluated according to the final diagnosis. Comparison between tests was obtained by examining the areas under the receiver operating characteristic curves. Results Among 155 patients, the etiology was biliary in 52% and alcoholic in 20%. The accuracy of the first US alone and of the 2 combined examinations for a biliary etiology were 66% and 83%, respectively. Comparison of receiver operating characteristic curves showed a better performance of repeated US (difference between areas under the curve, 0.135; 95% confidence interval, 0.02–0.24; P = 0.021). Magnetic resonance cholangiopancreatography had high specificity (93%) but low sensitivity (62%), with 76% accuracy. The accuracy of the combination of the 2 US examinations and of elevated alanine transferase was 87%. Conclusions Repeated US is effective for biliary AP diagnosis. The combination of repeated US examinations and biochemical tests seems an effective approach, whereas magnetic resonance cholangiopancreatography might be restricted to selected cases.

OBJECTIVES: The objective of this study was to determine the impact of different metastatic spread patterns on outcome in advanced digestive neuroendocrine tumors (NETs). METHODS: This was a retrospective analysis of patients with stage IV NETs, classified as group 1 (unilobar liver metastases), group 2 (bilobar liver metastases), group 3 (extra-abdominal metastases). End points were overall survival (OS) and progression-free survival (PFS). Risk factor analysis was performed using Cox proportional hazard model. RESULTS: Of the 229 patients, 135 (58.9%) had pancreatic, and 94 (41.1%) small bowel NETs: 32 (13.9%) were included in group 1, 179 (78.2%) in group 2, and 18 (7.9%) in group 3. Median Ki67 was 4.5%. Overall, 5-year OS was 55%. Different OS was observed among the 3 groups: median survival not reached, 81 and 8 months, respectively (P < 0.001). Median PFS was 18 months. Both OS and PFS were significantly affected by Ki67 and metastatic spread pattern. CONCLUSIONS: The stratification of stage IV NET patients based on metastatic patterns, alongside Ki67, predicts the clinical outcome. The extent of metastatic disease is a previously unrecognized variable, which should be considered when evaluating the results of treatments in NET patients with advanced disease.

Pancreatic cystic lesions (PCLs) are commonly diagnosed incidentally and are often preneoplastic. Their presence may influence the management of patients with chronic diseases such as liver cirrhosis (LC). This study evaluated the prevalence and nature of PCLs in LC patients.The images of 192 LC patients and 192 controls who underwent either computed tomography scan or nuclear magnetic resonance were reviewed for the presence and nature of PCLs. The prevalence of PCLs in both groups and differences between LC patients with and without PCLs were analysed. Multiple logistic regression analysis was used to investigate factors associated with PCLs.Thirty-five of 192 LC patients (18%) and 19/192 controls (10%) had PCLs (PCLs are more common in LC patients than in controls, and most are IPMNs. The occurrence of PCLs in LC patients seems to be related to age and disease duration.

Context Functional imaging tests (FITs) detecting somatostatin receptor expression [i.e., somatostatin receptor scintigraphy, 68Ga-DOTA positron emission tomography/computed tomography (CT)] have a pivotal role in the diagnosis of neuroendocrine tumors (NETs), although their indication during follow-up still needs to be clarified. Objective Investigate the role of FITs after diagnosis of metastatic enteropancreatic NETs, identifying patients who might benefit from these exams. Design Multicenter retrospective analysis of metastatic enteropancreatic NETs. Setting Analysis of imaging tests performed between January 1995 and December 2015 in Rome, Berlin, Milan, Marburg, or Graz. Subjects One hundred forty-three patients with metastatic pancreatic NETs and small intestine NETs, at least 2-year follow-up, and positive FITs. Interventions Patients had received CT every 6 months (unless clinical conditions and tumor behavior required shorter intervals) and FIT every 12 months. Main Outcome Measures Clinical usefulness of FITs, defined as changes in patient management (indication to biopsy, medical therapy, surgery, or further imaging tests) due only to FITs. Results FITs affected management in 73.4% of patients, mostly when G2 vs G1 [odds ratio (OR), 2.40; 95% confidence interval (CI), 1.09 to 5.27; P = 0.03]. Changes were observed in a 12-month time frame especially with pancreatic NETs vs small intestine NETs (OR, 2.89; 95% CI, 1.09 - 7.67; P = 0.03) or metastases since diagnosis vs developed during follow-up (OR, 4.00; 95% CI, 1.43 to 11.17; P < 0.01). Conclusions FITs used in addition to CT in the follow-up of stage IV enteropancreatic NETs improve patient management (especially for G2 tumors). Follow-up program should be tailored according to tumor features.

The imaging findings of a gastrointestinal stromal tumour (GIST) have been investigated in various studies; however, the features and the pattern of growth of an oesophageal GIST have been described only in a few reports. We present two cases studied by multidetector computed tomography (MDCT) and we review the literature. We describe the diagnostic course and the pattern of presentation at MDCT of two GISTs arising from the oesophageal wall that show two different behaviours and prognosis. We review the literature comparing the features of an oesophageal GIST to the more frequent gastric or intestinal GISTs, evaluating the role of MDCT in the diagnosis and in the follow-up. Oesophageal GISTs are uncommon, with less than 5% of all reported cases originating from this site of the GI tract. In our experience, the CT features of the two oesophageal GISTs appeared comparable to the imaging findings of the gastrointestinal stromal tumours of the stomach and of the small bowel, as these neoplasms show signs and a pattern of growth that are fairly characteristic. As reported in literature and appear in our experience, MDCT has an important role in the diagnostic course and in staging the disease even if a definitive diagnosis can be only made with the support of an immunohistochemical examination. In addition, MDCT is extremely useful in monitoring patients surgically or pharmacologically treated in order to evaluate the response to the therapy and the possibility of a progression of the disease.

Background.Renal cell carcinoma accounts for about 2-3% of all malignant tumors. The prevalence of brain metastases from RCC is less than 20% of cases. Traditionally, whole brain radiotherapy as well as the latest stereotactic radiosurgery improves both survival and local tumor control. These treatments also allow stabilization of clinical symptomatology. However, validated treatment guidelines for RCC patients with brain metastases are not yet available on account of the frequent exclusion of such patients from clinical trials. Moreover, limited data about the sequential use of three therapies, changing the class of agent, have been published up to now.Case Report.We report the case of a patient with metastatic RCC who developed disease progression after sunitinib and everolimus as first-line and second-line therapy, respectively. Thus, he underwent a multimodality treatment with pazopanib, as third-line therapy, to control systemic disease and radiosurgery directed on the new brain metastasis. To date, the patient is still receiving pazopanib, with progression-free survival and overall survival of 43 and 103 months, respectively.Conclusion.In a context characterized by different emerging options, with no general consensus on the optimal treatment strategy, the use of pazopanib in pretreated patients could be a suitable choice.

Background Knowledge of factors able to predict the clinical outcome of homogenous series of entero-pancreatic endocrine tumours treated with somatostatin analogues is poor. This study was aimed at identifying predictors for efficacy of somatostatin analogues at inhibiting tumour growth and modifying patients' survival during long-term follow-up. Patients and methods 31 patients with entero-pancreatic well-differentiated endocrine carcinoma received long-acting somatostatin analogues. All had progressive, metastatic disease (87% liver metastases, 38.7% distant extra-hepatic metastases). Results Response rate after 6 months of treatment was 45.2% (all disease stabilisation: 27.8% of pancreatic vs. 81.8% of intestinal tumours, P = 0.007). The predictors for non-response were: pancreatic tumour (OR 5.8), no previous surgery (OR 6.7), and the presence of distant extra-hepatic metastases, the latter being also confirmed by multivariate analysis (OR 10.0). Responders maintained stabilisation for 26.5 months, and none died during follow-up. Different survival curves were observed for patients, responding at 6 months compared to non-responders (P = 0.004), 3-year survival rate being 100% and 52.3%, respectively. Conclusions Distant extra-hepatic metastases are the major predictor of poor efficacy of somatostatin analogues in progressive, metastatic, well-differentiated entero-pancreatic endocrine carcinomas. Patients achieving response after 6 months of treatment, maintain it throughout a long-term follow-up. Non-responders after 6 months of treatment, have a worse survival, and should be considered for alternative treatments.

Celiac Disease and CFTR Mutations in Patients With Chronic Asymptomatic Pancreatic Hyperenzymemia

s / Digestive and Liver Disease 47S (2015) e43–e66 e63 driving the synthesis of abnormal peptides, possibly leading to virion assembly defects and to the intracytoplasmatic accumulation of HBsAg. Conclusions Selective pressure by the immune system selects variants in theprecore region that arenotproportionally detectable in the periphery, possibly due to the abnormal release of defective virions. This may also have clinical implications for hepatic pathology (steatosis) andmay lead to the underestimation of viral load. A better comprehensionof the relationshipbetween intrahepatic and peripheral (virological and clinical) markers may confer to these a higher diagnostic power. http://dx.doi.org/10.1016/j.dld.2015.01.136

P0367 SARCOPENIA IS ASSOCIATED WITH A REDUCED SURVIVAL IN PATIENTS WITH HEPATOCARCINOMA UNDERGOING SORAFENIB TREATMENT E. Gigante, G. Antonelli, P. Begini, F. Carbonetti, E. Iannicelli, P. Marchetti, A. Pellicelli, L. Miglioresi, G. Delle Fave, M. Marignani. Digestive and Liver Diseases unit, Radiology unit, Oncology unit, St. Andrea Hospital, Faculty of Medicine and Psychology, University ’Sapienza’, Liver Unit, San Camillo Forlanini Hospital, Rome, Italy E-mail: elia.gigante@hotmail.it