Linfoma di Burkitt in età pediatrica

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. <br> 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. <br> 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.

Unresected pediatric myoepithelial carcinomas treated with alternating chemotherapy cycles with adjuvant radiotherapy

ABSTRACTMyoepithelial carcinomas of soft tissue origin are rare tumors in the pediatric population. Due to the rarity of this malignancy, very few cases have been reported in the literature, and a consensus on treatment has not been established. Most myoepithelial carcinomas of soft tissue present in the extremities and are treated with surgical excision followed by adjuvant radiotherapy. We report 2 cases of pediatric myoepithelial carcinoma presenting with vertebral involvement making complete surgical removal impossible. These patients underwent chemotherapy and adjuvant radiotherapy as the main treatment for their primary tumors.