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Non-Hodgkin lymphoma is the fourth most common malignancy in children, has an even higher incidence in adolescents, and is primarily represented by only a few histologic subtypes. Dramatic progress has been achieved, with survival rates exceeding 80%, in large part because of a better understanding of the biology of the different subtypes and national and international collaborations. Most patients with Burkitt lymphoma and diffuse large B-cell lymphoma are cured with short intensive pulse chemotherapy containing cyclophosphamide, cytarabine, and high-dose methotrexate. The benefit of the addition of rituximab has not been established except in the case of primary mediastinal B-cell lymphoma. Lymphoblastic lymphoma is treated with intensive, semi-continuous, longer leukemia-derived protocols. Relapses in B-cell and lymphoblastic lymphomas are rare and infrequently curable, even with intensive approaches. Event-free survival rates of approximately 75% have been achieved in anaplastic large-cell lymphomas with various regimens that generally include a short intensive B-like regimen. Immunity seems to play an important role in prognosis and needs further exploration to determine its therapeutic application. ALK inhibitor therapeutic approaches are currently under investigation. For all pediatric lymphomas, the intensity of induction/consolidation therapy correlates with acute toxicities, but because of low cumulative doses of anthracyclines and alkylating agents, minimal or no long-term toxicity is expected. Challenges that remain include defining the value of prognostic factors, such as early response on positron emission tomography/computed tomography and minimal disseminated and residual disease, using new biologic technologies to improve risk stratification, and developing innovative therapies, both in the first-line setting and for relapse., (© 2015 by American Society of Clinical Oncology.)

Unlabelled: Cervical lymphadenopathy is a common disorder in children due to a wide spectrum of disorders. On the basis of a complete history and physical examination, paediatricians have to select, among the vast majority of children with a benign self-limiting condition, those at risk for other, more complex, diseases requiring laboratory tests, imaging and, finally, tissue sampling. At the same time, they should avoid expensive and invasive examinations when unnecessary. The Italian Society of Preventive and Social Pediatrics, jointly with the Italian Society of Pediatric Infectious Diseases, the Italian Society of Pediatric Otorhinolaryngology, and other Scientific Societies, issued a National Consensus document, based on the most recent literature findings, including an algorithm for the management of cervical lymphadenopathy in children., Methods: The Consensus Conference method was used, following the Italian National Plan Guidelines. Relevant publications in English were identified through a systematic review of MEDLINE and the Cochrane Database of Systematic Reviews from their inception through March 21, 2014., Results: Basing on literature results, an algorithm was developed, including several possible clinical scenarios. Situations requiring a watchful waiting strategy, those requiring an empiric antibiotic therapy, and those necessitating a prompt diagnostic workup, considering the risk for a severe underling disease, have been identified., Conclusion: The present algorithm is a practice tool for the management of pediatric cervical lymphadenopathy in the hospital and the ambulatory settings. A multidisciplinary approach is paramount. Further studies are required for its validation in the clinical field.

Objective: Children with leukaemia experience special difficulties adapting to stressful medical procedures and to the adverse effects of chemotherapy, though they can implement their coping strategies. The aims of the study were to assess whether the coping‐with‐pain strategies could be influenced by a child's personal and illness factors and to render possible comparisons between children with leukaemia and healthy peers. Another aim was to compare parents' and children's reports on coping strategies. Methods: A total of 125 patients (average age = 6.79 years; SD = 3.40) with acute leukaemia (lymphocytic leukaemia 90.4% and myeloid leukaemia 9.6%) and age‐matched healthy children with their parents were enrolled in the study. A socio‐demographic questionnaire and the Waldon–Varni Pediatric Pain Coping Inventory, parent and self‐report versions, were administered 1 month after diagnosis. Data regarding the therapy's side effects were recorded. Results: The comparison between proxy‐reports of the two groups of parents found significant differences in terms of social support, self‐cognitive instructions and catastrophising strategies. Children aged 6–10 years relied more heavily on distraction than children of other ages, using more problem‐solving and self‐cognitive instructions. The results indicated moderate parent–child agreement. Conclusion: Health professionals could help paediatric leukaemic patients in adopting more efficiently pain coping strategies applicable for different ages. [ABSTRACT FROM AUTHOR]

To describe incidence, causes, and outcomes related to pediatric intensive care unit (PICU) admission for patients undergoing hematopoietic stem cell transplantation (HSCT), we investigated the risk factors predisposing to PICU admission and prognostic factors in terms of patient survival. From October 1998 to April 2015, 496 children and young adults (0 to 23 years) underwent transplantation in the HSCT unit. Among them, 70 (14.1%) were admitted to PICU. The 3-year cumulative incidence of PICU admission was 14.3%. The main causes of PICU admission were respiratory failure (36%), multiple organ failure (16%), and septic shock (13%). The overall 90-day cumulative probability of survival after PICU admission was 34.3% (95% confidence interval, 24.8% to 47.4%). In multivariate analysis, risk factors predisposing to PICU admission were allogeneic HSCT (versus autologous HSCT, P  = .030) and second or third HSCT ( P  = .018). Characteristics significantly associated with mortality were mismatched HSCT ( P  = .011), relapse of underlying disease before PICU admission ( P  < .001), acute respiratory distress syndrome at admission ( P  = .012), hepatic failure at admission ( P  = .021), and need for invasive ventilation during PICU course ( P  < .001). Our data indicate which patients have a high risk for PICU admission after HSCT and for dismal outcomes after PICU stay. These findings may provide support for the clinical decision-making process on the opportunity of PICU admission for severely compromised patients after HSCT. [ABSTRACT FROM AUTHOR]

We explored the feasibility of unrelated donor haematopoietic stem cell transplant (HSCT) upfront without prior immunosuppressive therapy (IST) in paediatric idiopathic severe aplastic anaemia (SAA). This cohort was then compared to matched historical controls who had undergone first-line therapy with a matched sibling/family donor (MSD) HSCT ( n = 87) or IST with horse antithymocyte globulin and ciclosporin ( n = 58) or second-line therapy with unrelated donor HSCT post-failed IST ( n = 24). The 2-year overall survival in the upfront cohort was 96 ± 4% compared to 91 ± 3% in the MSD controls ( P = 0·30) and 94 ± 3% in the IST controls ( P = 0·68) and 74 ± 9% in the unrelated donor HSCT post-IST failure controls ( P = 0·02).The 2-year event-free survival in the upfront cohort was 92 ± 5% compared to 87 ± 4% in MSD controls ( P = 0·37), 40 ± 7% in IST controls ( P = 0·0001) and 74 ± 9% in the unrelated donor HSCT post-IST failure controls ( n = 24) ( P = 0·02). Outcomes for upfront-unrelated donor HSCT in paediatric idiopathic SAA were similar to MSD HSCT and superior to IST and unrelated donor HSCT post-IST failure. Front-line therapy with matched unrelated donor HSCT is a novel treatment approach and could be considered as first-line therapy in selected paediatric patients who lack a MSD. [ABSTRACT FROM AUTHOR]

A prospective pediatric survey on the incidence of central venous catheter (CVC) complications was performed aimed at identifying risk factors of premature CVC removal. The study comprised 129 Broviac-Hickman CVCs inserted during a 13-month period in 112 children. The total number of CVC days was 19,328 (median: 122 days, range: 1–385). The overall rate of complications was 6.2/1000 CVC days, i.e., 4.5/1000 and 1.7/1000 CVC days for mechanical and infectious complications, respectively. Interestingly, only two CVC-related cases of septicemia and no thrombotic events were documented. At the end of the study period, 38 of 129 CVC (29.5%) had been removed: 20 due to CVC-related complications (dislocation18, rupture 2), 10 due to the patient’s death, and 8 due to completion of therapy. Age at CVC insertion <4.9 years was a significant predictor of premature CVC removal (p=0.01). Mechanical complications, especially in younger children, are the main cause of premature loss of CVC. These data underline the importance of more effectively securing the CVC to subcutaneous tissue in pediatric patients to reduce accidental dislocations. [ABSTRACT FROM AUTHOR]

Summary. This study aimed to ascertain whether extracorporeal photochemotherapy (ECP) is an effective treatment for paediatric patients with refractory graft-versus-host disease (GVHD). From January 1992 to December 2000, 77 children (median age 8·6 years) with either acute (n = 33) or chronic (n = 44) GVHD, resistant to conventional immunosuppressive therapy, were treated with ECP in four Italian paediatric hospitals. After ECP, acute GVHD involving skin, liver and gut responded completely in 76%, 60% and 75% of patients respectively. The 5-year overall survival was 69% for responding patients vs 12% for non-responders (P = 0·001). Among the 44 children with chronic GVHD, 15 (44%) showed a complete response and 10 (29%) a significant improvement after ECP. The 5-year overall survival was 96% for responders vs 58% for non-responders (P = 0·04). Our results suggest that ECP is an effective treatment that may be useful in paediatric patients with either acute or chronic GVHD who have failed to respond to standard immunosuppressive therapy. [ABSTRACT FROM AUTHOR]

Abstract: Rib tumors are uncommon and represent 5% to 10% of all bony tumors. Regarding the benign rib lesions, costal angioma is very rare in childhood. We report a case of a rare angioma of a rib complicated by bone erosion in a young boy. [Copyright &y& Elsevier]