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Sporadic Burkitt lymphoma (BL) accounts for about 80% of non-Hodgkin lymphomas in childhood and adolescence, with an incidence of 3-5 cases per million in developed countries. The hallmark of LB is the deregulation of MYC oncogene, usually caused by the [t(8;14)(q24;q32)] translocation, involving the gene of immunoglobulin heavy chain. BL is a very aggressive neoplasm, with a doubling time of 12-24 hours and its diagnosis and therapy are a true haematologic emergency. Abdomen, head and neck are the most frequent sites of presentation and the involvement of ileo-caecal area can cause an intussusception as first manifestation of the disease. BL and mature B-cell acute leukaemia (the leukaemic manifestation of BL) share the same therapeutic approach, intensive short courses with multiple alternating chemotherapy agents, the use of high dose-methotrexate and intrathecal CNS prophylaxis. The outcomes are satisfactory: in Italy during the period 1997-2014 the children with BL were treated according to the AIEOP LNH-97 protocol, achieving more than 90% of 5 year-survival. In the last decades, the prognosis for children and adolescents with cancer has dramatically improved and the increasing number of long-term survivors has focused the attention of clinicians on the late effects of cancer and its therapy. A multidisciplinary follow up and a correct transition from the paediatrician to the primary care doctor plays a central role not only in the early detection and therapy of late effects of cancer but also in supporting the patient to realize their physical, mental and social well-being as much as possible.

Treatment intensification was considered a suitable strategy to increase the cure rate of lymphoblastic lymphoma (LBL) in children.The AIEOP LNH-97 trial was run between 1997 and 2007 for newly diagnosed LBL in patients aged less than 18 years. Treatment schedule was based on the previous, LSA2-L2 derived, AIEOP LNH-92 protocol. Modifications included: increased dose of upfront cyclophosphamide and methotrexate, use of l-Asparaginase during induction therapy, intensive block therapy for slow responders, and late intensification ("Reinduction") for patients with advanced stage disease. Total therapy duration was 12 months for stage I and II, and 24 months for stage III and IV. Central nervous system prophylaxis did not include cranial irradiation.114 eligible patients were enrolled, 84 males and 30 females; median age was 9 years. Complete remission was obtained in 98% of patients. After a median follow-up time of seven years, 29 patients failed due to progression of disease (n = 2), relapse (n = 25), or second malignancy (n = 2). The 7-year overall survival was 82% (standard error [SE] 4%) and the 7-year event-free survival was 74% (SE 4%). No subgroup showed significantly different event free survival. None of the patients died of front line chemotherapy-related toxicity.Treatment intensification was associated with good outcome in children and adolescents with LBL, with limited toxicity. Prognosis after relapse was better for patients who underwent allogeneic hematopoietic stem cell transplantation. Measurements of biological markers and treatment response are necessary for achieving further improvement through more accurate identification and stratification of patients at risk of disease relapse.

Lymphoblastic lymphoma (LBL) is the second most frequent lymphoma subtype in childhood. It is commonly treated according to therapy strategies for lymphoblastic leukemia.The AIEOP LNH-92 protocol was a modified LSA2-L2 therapy used for both T- and B-cell precursor LBL and included Induction, Consolidation, and Maintenance treatment with a total duration of 11 and 24 months for stages I and II, stages III and IV disease, respectively.Fifty-five eligible patients were enrolled, 40 males and 15 females, with a median age of 8 years. Complete remission was achieved in 93% of the cases. With a median follow-up of 9 years the event-free survival (EFS) was 69% and overall survival 72%. EFS of localized disease was 100%. The most frequent grades III and IV toxicity was hematologic and hepatic (elevated transaminases) toxicity. No toxic death nor second tumor were observed. Outcome was comparable to most concomitant international protocols for LBL, but inferior to recent trials that included reinduction treatment or a higher intensity therapy for high stage disease.AIEOP LNH92 protocol demonstrated similar efficacy compared to contemporary regimens, with limited toxicity. Nevertheless, an intensified treatment is warranted for high stage disease.

Invasive aspergillosis is an uncommon but often lethal complication in immunocompromised patients. Despite the progress obtained with new antifungal drugs, intracranial aspergillosis often requires a combined medical and surgical approach. Most cases previously reported in immunocompromised children were fatal. We describe 4 immunosuppressed children with intracranial aspergillosis successfully treated with surgery and antifungal long-term therapy.

Desferrioxamine (DFO) has shown anti-proliferative and cytotoxic effects on several tumor cells. DFO is used at present in the treatment of neuroblastoma in combination with chemotherapy (D-CECaT regimen: cyclophosphamide, etoposide, carboplatin, and thiotepa). We compared the effect of continuous or intermittent exposures to DFO on 3H-thymidine uptake, viability, and cell cycle of human neuroblastoma (NB) cell lines. Our results show that continuous exposures to DFO cause dose- and time-dependent cytotoxicity of NB cells, while intermittent exposures result in significant NB cell toxicity only when using high DFO concentrations. By 3H-thymidine uptake, a significant inhibition of proliferation was observed only in continuous exposures. In addition, a consistent arrest in G1 phase was detected only in cultures treated continuously with high DFO concentrations. Our data indicate that 3H-thymidine uptake, viability, and cell cycle changes are proportional to the extent of exposure and concentration of DFO, suggesting that in vivo DFO continuous infusion may improve anti-neuroblastoma activity.