Abstract
Non-Hodgkin lymphoma (NHL) is the third most common childhood malignancy; peak incidence is between 7 and 10 years. A male predominance is evident.
Unlike adult lymphomas, childhood NHL more often presents as high-grade fast-growing tumour. Burkitt lymphoma is the most common form.
Many children present with disseminated disease and extranodal involvement, including bone marrow invasion or/and malignant effusions. Early diagnosis and referral to the specialized pediatric cancer centre is crucial to successful treatment.
Accurate histological classification is essential for allocating patients to appropriate treatment groups. Main therapy groups are lymphoblastic lymphoma, mature B-cell lymphoma, and anaplastic large cell lymphoma.
Intensive combined chemotherapy is primary treatment modality in childhood NHL. For CNS-negative patients, treatment includes intrathecal methotrexate (MTX) and systemic high-dose MTX (1-5 g/m2) administration.
With current therapies cure rates range from 70% to over 90%, even for disseminated disease. Highly efficacious regimens have considerable acute toxicity, patients with disseminated NHL have 3-5% toxic death risk.
Future treatment strategies include improved risk classification, the investigation of targeted therapy, and an increased understanding of the biology of childhood NHL.