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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.

Il contributo descrive l’attività del Gruppo Protezione Civile Legambiente Beni Culturali Marche. Nella prima parte, dopo aver fornito qualche elemento in merito al ruolo del volontariato specializzato nella messa in sicurezza del patrimonio culturale in situazioni di emergenza e definita la funzione che il Gruppo ha avuto negli ultimi venti anni, si focalizza l’attenzione sull’esperienza aquilana e sul modello operativo gestionale messo a punto e consolidato durante quell’emergenza. La seconda parte, dedicata all’emergenza Sisma Centro Italia 2016, fornisce un primo e provvisorio bilancio delle attività svolte e di quelle ancora in corso e riflette sugli aspetti del sistema emergenziale che hanno funzionato e su quanto andrebbe ripensato. L’ultima parte propone una serie di spunti che intendono considerare l’emergenza quale punto di partenza per la costruzione di nuovi modelli operativi. The paper describes the activity of Gruppo Protezione Civile Legambiente Beni Culturali Marche. After clarifying the role of professional volunteering in the protection of cultural heritage during emergency and defining the Group’s function in the last twenty years, the first part focuses on L’Aquila experience and on the connected operational model. In the second part, the paper focuses on 2016 Central Italy earthquake, providing a provisional report of the work that has been already done and of in progress activities and reflecting on strengths and weaknesses of the emergency management system. In the final part it points out that emergency can be considered an opportunity to create new operational models.