Incidence and clinicopathologic features of gastrointestinal stromal tumors. A population-based study.

BACKGROUND: Although the diagnostic criteria and pathogenesis of gastrointestinal stromal tumors (GIST) have recently been elucidated, knowledge of the epidemiology of this malignancy is still limited. This study examined the incidence of GIST in the province of Modena, including pathologic features and clinical outcome. METHODS: Gastrointestinal mesenchymal tumors identified by the Modena Cancer Registry between 1991 and 2004 were analyzed with an immunohistochemical panel that included staining for CD-117 and PDGFRalpha. Size, mitotic rate, and other pathologic parameters were recorded. Each tumor was categorized into National Institutes of Health risk categories (very low, low, intermediate, and high risk). RESULTS: One hundred twenty-four cases were classified as GIST. The age-adjusted incidence rate was 6.6 per million. Seventy-five percent of patients were symptomatic; 34% had a previous or concomitant history of cancer. High-risk features were present in 47% of cases. Seventy-eight percent were submitted to radical surgery. After complete resection, the 5-year disease-free survival rates were 94%, 92%, 100%, and 40% for patients at very low, low, intermediate, and high risk, respectively. In multivariate analysis, high risk was the main predictor of recurrence. CONCLUSION: This population-based study shows that the incidence of GIST in Northern Italy is comparable to that reported in other European countries. Survival was favorable in lower risk categories and in most of the resected cases. In our study, resected patients at very low, low, and intermediate risk had a similar outcome. Our data support the need to consider high-risk patients after complete surgical resection for treatment with the best available approach

Carcinoid Crisis: A Misunderstood and Unrecognized Oncological Emergency

Carcinoid Crisis represents a rare and extremely dangerous manifestation that can occur in patients with Neuroendocrine Tumors (NETs). It is characterized by a sudden onset of hemodynamic instability, sometimes associated with the classical symptoms of carcinoid syndrome, such as bronchospasm and flushing. Carcinoid Crisis seems to be caused by a massive release of vasoactive substances, typically produced by neuroendocrine cells, and can emerge after abdominal procedures, but also spontaneously in rare instances. To date, there are no empirically derived guidelines for the management of this cancer-related medical emergency, and the available evidence essentially comes from single-case reports or dated small retrospective series. A transfer to the Intensive Care Unit may be necessary during the acute setting, when the severe hypotension becomes unresponsive to standard practices, such as volemic filling and the infusion of vasopressor therapy. The only effective strategy is represented by prevention. The administration of octreotide, anxiolytic and antihistaminic agents represents the current treatment approach to avoid hormone release and prevent major complications. However, no standard protocols are available, resulting in great variability in terms of schedules, doses, ways of administration and timing of prophylactic treatments

Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a group of systemic vasculitides that predominantly affect small vessels, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Pulmonary involvement is frequently observed in AAV patients, with various possible phenotypes in the different diseases. In the last years, among the possible types of lung involvement, a growing interest has been addressed to the interstitial lung disease (ILD). Prevalence of ILD is higher in MPA than in GPA; in fact, ILD has been reported in up to 45% of MPA patients and in 23% of GPA. Anti-MPO antibodies are the main ANCA subtype associated to ILD, in about 46-71% of cases, while anti-PR3 antibodies are reported in 0-29% of patients. High resolution computed tomography (HRCT) frequently detects interstitial lung abnormalities in AAV, up to 66% of patients with MPA, even if with an unclear clinical relevance, specifically in asymptomatic patients. Ground glass opacities, mainly consistent with diffuse alveolar hemorrhage (DAH), are the most frequent finding in MPA patients, but reticulations, interlobular septal thickening and honeycombing are also reported. ILD significantly affects quality of life and survival, with mortality increased 2 to 4 times, particularly higher in MPA patients with pulmonary fibrosis. Currently, immunosuppressive therapy is considered also as a possible treatment of ILD. However, a careful evaluation of progression and severity of lung involvement, should guide the treatment decision in the single patient. In this review, we discuss the available evidence on clinical features, diagnostic work-up, prognosis and management of AAV-ILD

Role of Notch2 pathway in mature B cell malignancies

In recent decades, the Notch pathway has been characterized as a key regulatory signaling of cell-fate decisions evolutionarily conserved in many organisms and different tissues during lifespan. At the same time, many studies suggest a link between alterations of this signaling and tumor genesis or progression. In lymphopoiesis, the Notch pathway plays a fundamental role in the correct differentiation of T and B cells, but its deregulated activity leads to leukemic onset and evolution. Notch and its ligands Delta/Jagged exhibit a pivotal role in the crosstalk between leukemic cells and their environment. This review is focused in particular on Notch2 receptor activity. Members of Notch2 pathway have been reported to be mutated in Chronic Lymphocytic Leukemia (CLL), Splenic Marginal Zone Lymphoma (SMZL) and Nodal Marginal Zone Lymphoma (NMZL). CLL is a B cell malignancy in which leukemic clones establish supportive crosstalk with non-malignant cells of the tumor microenvironment to grow, survive, and resist even the new generation of drugs. SMZL and NMZL are indolent B cell neoplasms distinguished by a distinct pattern of dissemination. In SMZL leukemic cells affect mainly the spleen, bone marrow, and peripheral blood, while NMZL has a leading nodal distribution. Since Notch2 is involved in the commitment of leukemic cells to the marginal zone as a major regulator of B cell physiological differentiation, it is predominantly affected by the molecular lesions found in both SMZL and NMZL. In light of these findings, a better understanding of the Notch receptor family pathogenic role, in particular Notch2, is desirable because it is still incomplete, not only in the physiological development of B lymphocytes but also in leukemia progression and resistance. Several therapeutic strategies capable of interfering with Notch signaling, such as monoclonal antibodies, enzyme or complex inhibitors, are being analyzed. To avoid the unwanted multiple “on target” toxicity encountered during the systemic inhibition of Notch signaling, the study of an appropriate pharmaceutical formulation is a pressing need. This is why, to date, there are still no Notch-targeted therapies approved. An accurate analysis of the Notch pathway could be useful to drive the discovery of new therapeutic targets and the development of more effective therapies

Human herpesvirus 8-associated primary effusion lymphoma in human immunodeficiency virus-negative patients: a clinico-epidemiologic variant resembling classic Kaposi's sarcoma

No abstract availabl

Evaluation of carcass quality, body and pulmonary lesions detected at the abattoir in heavy pigs subjected or not to tail docking

BackgroundNowadays, body and tail lesions and respiratory disease are some of the greatest problems affecting the health and welfare of pigs. The aim of the study was to measure the prevalence of pleurisy, bronchopneumonia (enzootic pneumonia like lesions) and lesions on tail and body of heavy pigs subjected or not to tail docking through the inspection in Italian abattoirs. Additionally, the effect of tail docking and season was investigated on carcass quality (weight, % of lean meat, and Protected Designation of Origin (PDO) classification). For this purpose, a total 17.256 carcasses belonging to 171 batches from 103 farms were inspected in an Italian abattoir between 2019 and 2022. Enzootic pneumonia (EP) like lesions were scored according to the Madec and Derrien method, while pleurisy was scored using the Italian Slaughterhouse pleuritic evaluation system (SPES). For the tail and body, the lesions were scored according to Welfare Quality. The lesion score index (LSI) was calculated for each area. Data were analysed using a general linear model (GLM) including tail caudectomy, season and distance of the farm from the abattoir.ResultsThe warm season increased the percentage of lesions in carcasses in all parts of the body observed (P < 0.0001). The presence of undocked tail increased the LSI of the tail (P < 0.0001). The percentage of limbs lesions with score 2 and limbs LSI increase with increasing duration of transport (coef. = 0.003, P < 0.001; coef. = 0.008, P < 0.001; respectively). The hot carcass weight and the percentage of carcasses included in the PDO were higher in batches with docked tails (P = 0.027; P < 0.001, respectively), while the percentage of lean meat was higher in batches with undocked tails (P < 0.001). There was a negative correlation between the percentage of carcasses included in PDO and the LSI of tail (r = - 0.422; P < 0.001).ConclusionsIn conclusion, the presence of the undocked tail and the warm season can be considered risk factors for the prevalence of tail lesions, while long transport can increase limb lesions. Furthermore, the carcass weight and meat quality were negatively influenced by tail lesions

Novel plasmid-mediated colistin resistance mcr-4 gene in Salmonella and Escherichia coli, Italy 2013, Spain and Belgium, 2015 to 2016

A novel mcr colistin resistance gene was identified in a strain of Salmonella enterica, monophasic variant of serovar Typhimurium (4,5,12:i:- ), isolated from a pig at slaughter in Italy in 2013, and in Escherichia coli strains collected during routine diagnostic of postweaning diarrhoea in pigs from Spain and Belgium in 2015 and 2016. Immediate implementation of mcrscreening including this novel gene variant is required for Salmonella and E. coli from humans and food-producing animals in Europe. © 2017, European Centre for Disease Prevention and Control (ECDC). All rights reserved