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Stage 4?s neuroblastoma: features, management and outcome of 268 cases from the Italian Neuroblastoma Registry.

ABSTRACT: BACKGROUND:Infants diagnosed with stage 4?s neuroblastoma commonly experience spontaneous disease regression, with few succumbing without response to therapy. We analyzed a large cohort of such infants enrolled in the Italian Neuroblastoma Registry to detect changes over time in presenting features, treatment and outcome. METHODS:Of 3355 subjects aged 0-18?years with previously untreated neuroblastoma diagnosed between 1979 and 2013, a total of 280 infants (8.3%) had stage 4?s characteristics, 268 of whom were eligible for analyses. Three treatment eras were identified on the basis of based diagnostic and chemotherapy adopted. Group 1 patients received upfront chemotherapy; Group 2 and 3 patients underwent observation in the absence of life-threatening symptoms (LTS), except for Group 3 patients with amplified MYCN gene, who received more aggressive therapy. RESULTS:The three groups were comparable, with few exceptions. Ten-year overall survival significantly increased from 76.9 to 89.7% and was worse for male gender, age 0-29?days and presence of selected LTS on diagnosis, elevated LDH, and abnormal biologic features. Infants who underwent primary resection ± chemotherapy did significantly better. On multivariate analysis, treatment eras and the association of hepatomegaly to dyspnea were independently associated with worse outcome. CONCLUSIONS:Our data confirm that stage 4?s neuroblastoma is curable in nearly 90% of cases. Hepatomegaly associated to dyspnea was the most important independent risk factor. The cure rate could be further increased through timely identification of patients at risk who might benefit from surgical techniques, such as intra-arterial chemoembolization and/or liver transplantation, which must be carried out in institutions with specific expertise.

SUBMITTER: De Bernardi B

PROVIDER: S-EPMC6329141 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature

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Stage 4 s neuroblastoma: features, management and outcome of 268 cases from the Italian Neuroblastoma Registry.

De Bernardi Bruno B Di Cataldo Andrea A Garaventa Alberto A Massirio Paolo P Viscardi Elisabetta E Podda Marta Giorgia MG Castellano Aurora A D'Angelo Paolo P Tirtei Elisa E Melchionda Fraia F Vetrella Simona S De Leonardis Francesco F D'Ippolito Carmelita C Tondo Annalisa A Nonnis Antonella A Erminio Giovanni G Gigliotti Anna Rita AR Mazzocco Katia K Haupt Riccardo R

Italian journal of pediatrics 20190111 1

<h4>Background</h4>Infants diagnosed with stage 4 s neuroblastoma commonly experience spontaneous disease regression, with few succumbing without response to therapy. We analyzed a large cohort of such infants enrolled in the Italian Neuroblastoma Registry to detect changes over time in presenting features, treatment and outcome.<h4>Methods</h4>Of 3355 subjects aged 0-18 years with previously untreated neuroblastoma diagnosed between 1979 and 2013, a total of 280 infants (8.3%) had stage 4 s cha ...[more]

PMID: 30634996

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Project description:Background Coronary artery disease was hitherto a rarity in Africa. Acute coronary syndrome (ACS) accounts for coronary artery disease-related morbidity and mortality. Reports on ACS in Africa are few. Methods and Results We enrolled 1072 indigenous Nigerian people 59.2±12.4 years old (men, 66.8%) with ACS in an observational multicentered national registry (2013-2018). Outcome measures included incidence, intervention times, reperfusion rates, and 1-year mortality. The incidence of ACS was 59.1 people per 100 000 hospitalized adults per year, and comprised ST-segment-elevation myocardial infarction (48.7%), non-ST-segment-elevation myocardial infarction (24.5%), and unstable angina (26.8%). ACS frequency peaked 10 years earlier in men than women. Patients were predominantly from urban settings (87.3%). Median time from onset of symptoms to first medical contact (patients with ST-segment-elevation myocardial infarction) was 6 hours (interquartile range, 20.1 hours), and only 11.9% presented within a 12-hour time window. Traditional risk factors of coronary artery disease were observed. The coronary angiography rate was 42.4%. Reperfusion therapies included thrombolysis (17.1%), percutaneous coronary intervention (28.6%), and coronary artery bypass graft (11.2%). Guideline-based pharmacotherapy was adequate. Major adverse cardiac events were 30.8%, and in-hospital mortality was 8.1%. Mortality rates at 30 days, 3 months, 6 months, and 1 year were 8.7%, 9.9%, 10.9%, and 13.3%, respectively. Predictors of mortality included resuscitated cardiac arrest (odds ratio [OR], 50.0; 95% CI, 0.010-0.081), nonreperfusion (OR, 34.5; 95% CI, 0.004-0.221), pulmonary edema (OR, 11.1; 95% CI, 0.020-0.363), left ventricular diastolic dysfunction (OR, 4.1; 95% CI, 0.091-0.570), and left ventricular systolic dysfunction (OR, 2.1; 95% CI, 1.302-3.367). Conclusions ACS burden is rising in Nigeria, and patients are relatively young and from an urban setting. The system of care is evolving and is characterized by lack of capacity and low patient eligibility for reperfusion. We recommend preventive strategies and health care infrastructure-appropriate management guidelines.

Dataset Information

Stage 4?s neuroblastoma: features, management and outcome of 268 cases from the Italian Neuroblastoma Registry.

Publications

Stage 4 s neuroblastoma: features, management and outcome of 268 cases from the Italian Neuroblastoma Registry.

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