Non-Hodgkin Lymphomas in Children and Adolescents
Non-Hodgkin lymphoma (NHL) is the fourth most common malignancy in children, with an even higher incidence in adolescents, and is primarily represented by only a few histological subtypes. Dramatic progress has been achieved, with survival rates exceeding 80%. Most patients with Burkitt lymphoma and diffuse large B-cell lymphoma are cured with short, intensive, pulse chemotherapy. The benefit of the addition of rituximab has been demonstrated for high-risk B-NHL and primary mediastinal B-cell lymphoma. Lymphoblastic lymphoma is treated with intensive, semi-continuous, longer ‘leukaemia-derived’ protocols. Relapses in B-cell and lymphoblastic lymphomas are rare and infrequently curable, even with intensive approaches. Event-free survival rates of about 75% have been achieved in anaplastic large-cell lymphomas with various regimens, including generally a short, intensive ‘B-like’ regimen. The role of immunity appears important in prognosis and needs further exploration in therapy. Anaplastic lymphoma kinase (ALK) inhibitor therapeutic approaches are currently being investigated. For all these paediatric lymphomas, the intensity of induction/consolidation treatments correlates with a high rate of immediate toxicities, but due to 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 (PET)/computed tomography (CT) and monitoring of minimal disseminated and residual disease, utilizing new biological technologies to improve risk stratification and the development of innovative therapies, both at frontline and relapse. non-Hodgkin lymphoma, NHL, European Intergroup for Childhood NHL, EICNHL, Burkitt lymphoma, anaplastic large-cell lymphoma, ALK, lymphoblastic lymphoma