Pediatric Lymphoma

Etiology and Epidemiology

The National Comprehensive Cancer Network recommends that pediatric aggressive mature B-cell lymphomas, including Burkitt lymphoma (BL) and diffuse large B-cell lymphoma (DLBCL), have specific etiologic associations according to their epidemiologic variant 1
The American Cancer Society notes that the endemic variant of Burkitt lymphoma is associated with Epstein-Barr virus (EBV) infection in approximately 95% of cases, primarily occurring in equatorial Africa, South America, Turkey, and Papua New Guinea, and is frequently associated with malaria infection 1, 2
The Centers for Disease Control and Prevention state that the immunodeficiency-associated variant of Burkitt lymphoma occurs mainly in individuals with HIV, where it can be the initial condition defining AIDS, with up to 70% of these patients positive for EBV 2, 3
The National Cancer Institute reports that the sporadic variant of Burkitt lymphoma is approximately 15% EBV-positive, primarily occurring in North America and Europe 3, 4

The American Academy of Pediatrics notes that the typical presentation of pediatric lymphoma is painless adenopathy, occurring in approximately 79% of cases, although manifestations vary significantly depending on tumor location 5
The National Comprehensive Cancer Network recommends that symptoms such as fever, unexplained weight loss, and profuse night sweats, known as "B" symptoms, are more common in advanced disease 5
The American Cancer Society states that fatigue or lethargy can result from bone marrow infiltration and subsequent cytopenias 5
The Centers for Disease Control and Prevention report that easy bruising or bleeding can occur due to thrombocytopenia secondary to bone marrow involvement 5

The National Comprehensive Cancer Network recommends that excisional or incisional biopsy of the most accessible site, with fresh tissue sent to pathology in saline solution, is preferred to ensure viable diagnostic tissue 6
The American College of Radiology notes that imaging studies, including CT scans and PET scans, can help identify tumor location and extent of disease 6

The National Comprehensive Cancer Network states that all pediatric lymphomas are highly aggressive but curable malignancies requiring complex treatment, and it is strongly preferred that treatment occurs in specialized centers with experience in managing these diseases 7, 5
The American Society of Clinical Oncology recommends that rituximab plus intensive chemotherapy is the standard of care for CD20-positive Burkitt lymphoma (CD20 present in 86-100% of cases) 8
The National Cancer Institute notes that systemic chemotherapy with intrathecal therapy is essential for preventing central nervous system (CNS) relapse in all patient categories 8

The American Cancer Society reports that 5-year survival rates have improved dramatically, now exceeding 95% for Hodgkin lymphoma and approximately 80-90% for non-Hodgkin lymphoma 5
The National Comprehensive Cancer Network states that the addition of rituximab to intensive chemotherapy regimens has significantly improved long-term outcomes, with event-free survival improving by approximately 20% in clinical trials 8