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BackgroundInfants 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.MethodsOf 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.ResultsThe 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.ConclusionsOur 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.Electronic supplementary materialThe online version of this article (10.1186/s13052-018-0599-1) contains supplementary material, which is available to authorized users.

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Ewing sarcoma (ES) is one of the most common pediatric solid tumors with aggressive behavior and unfavorable survival. In this study, we evaluated the diagnostic accuracy of baseline and restaging fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) scans and their possible prognostic role in pediatric ES. We evaluated 17 patients who underwent a total of 27 18F-FDG-PET/CT scans (10 for staging and 17 for restaging). The PET images were analyzed visually and semiquantitatively by measuring SUVmean, SUVmax, SUVlbm, SUVbsa, MTV, and TLG. Moreover, PET/CT results were compared with other conventional imaging (CI) results. Among 10 baseline PET/CT scan results, 9 were positive and 1 not valuable by interference; baseline PET/CT and CI were concordant in 7 cases and discordant in 2, with pulmonary micrometastases not detected by PET/CT. Among 17 restaging PET/CT scan results, 9 were positive and 8 negative; CI and restaging PET/CT were concordant in 9 cases and discordant in 8. Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of restaging 18F-FDG-PET/CT were 73%, 83%, 89%, 62.5%, and 76%, respectively. After a median follow-up of 20 months, relapse/progression occurred in 8 patients and death in 5. A positive 18F-FDG-PET/CT at restaging was significantly associated with shorter overall survival compared with unremarkable PET/CT at the same timepoint, but not with progression-free survival. Instead, metabolic PET/CT features were not correlated with outcome. 18F-FDG-PET/CT showed a good diagnostic performance in pediatric ES; except for pulmonary micrometastases, PET/CT was better than CI at restaging. Only restaging PET/CT result was significantly correlated with overall survival.

Summary: The aim of this study was to assess the efficacy of a solution composed by verbascoside, polyvinylpyrrolidone, and sodium hyaluronate (Mucosyte) in the treatment of chemotherapyinduced oral mucositi (OM). Patients between 5 and 18 years receiving chemotherapy for acute lymphoblastic leukemia and with OM grade 1 or 2 were randomized in group A (treated with Mucosyte, 3 mouthwashes/d per 8 d) and group B (treated with placebo, ie, an inert water-based solution, 3 mouthwashes/d per 8 d). The OM scoring was performed at day 1 (diagnosis of OM-T0), after 3 days of treatment (T1), and at day 8 (T2). Pain was evaluated through the visual analog scale with the same timing of OM measurement. A total of 56 patients were included (28 patients per group). Group A experienced a statistically significant decline of OM at T2 (P = 0.0038); a statistically significant difference in pain reduction between 2 groups both at T1 and at T2 (P < 0.005) was observed. The use of Mucosyte mouthwashes in children with chemotherapy-induced OM may be recommended as supportive therapy.