(See also Overview of Lymphoma.)
Classic Burkitt lymphoma is endemic in central Africa and constitutes 30% of childhood lymphomas in the US. The form endemic to Africa often manifests as enlargement of the jaw or facial bones.
In sporadic (non-African) Burkitt lymphoma, abdominal disease predominates, often arising in the region of the ileocecal valve or the mesentery. Tumor may cause bowel obstruction. Extranodal sites such as the brain or other solid organs may also be involved. In adults, disease may be bulky and generalized, often with massive involvement of liver, spleen, and bone marrow. Central nervous system (CNS) involvement is often present at diagnosis or with relapsing lymphoma.
Burkitt lymphoma is the most rapidly growing human tumor, and pathology reveals a high mitotic rate, a monoclonal proliferation of B cells, and a “starry-sky” pattern of benign macrophages that have engulfed apoptotic malignant lymphocytes. On FDG-PET (fluorodeoxyglucose-positron emission tomography) scans, the tumors are highly metabolic. There is a distinctive genetic translocation involving the C-myc gene on chromosome 8 and the immunoglobulin heavy chain of chromosome 14. The disease is closely associated with Epstein-Barr virus infection in endemic lymphoma; however, it is uncertain whether Epstein-Barr virus plays an etiologic role. Burkitt lymphoma occurs frequently in patients with HIV/AIDS and may be an AIDS-defining disease.
Histopathologic diagnosis is based on biopsy of lymph node or tissue from another suspected disease site, such as the bone marrow. Rarely, laparoscopy may be used for both diagnosis and treatment.
Staging includes FDG-PET/CT tumor imaging; if unavailable, CT of the chest, abdomen, and pelvis may be done instead. Patients should also have bone marrow biopsy, cerebrospinal fluid cytology, and laboratory studies to include lactate dehydrogenase (LDH). Staging studies must be expedited because of rapid tumor growth.
Treatment must be initiated rapidly because these tumors grow rapidly. An intensive alternating regimen of cyclophosphamide, vincristine, doxorubicin, methotrexate, ifosfamide, etoposide, and cytarabine (CODOX-M/IVAC) plus rituximab results in a cure rate of > 80% for children and adults < 60 years. For patients > 60 years, regimens such as rituximab plus etoposide, prednisone, vincristine (Oncovin), and doxorubicin (dose-adjusted R-EPOCH) are also commonly used with success. For patients without CNS metastases, CNS prophylaxis is essential.
With treatment, tumor lysis syndrome is common, and patients must receive IV hydration, allopurinol often with rasburicase, alkalinization, and close attention to electrolytes (particularly potassium, phosphorus, and calcium). Some patients may require dialysis for hyperkalemia.
If the patient presents with bowel obstruction secondary to tumor but the tumor is completely resected at initial diagnostic-therapeutic laparotomy, then aggressive therapy is still indicated, but fewer cycles may be needed. Patients at end of treatment should have a complete metabolic response documented by PET or a complete response documented by CT scan and bone marrow biopsy. Outcome is poor in the 20% of patients in whom induction fails or relapse (typically in the first 12 months) occurs. Salvage therapy or clinical trials should be considered.
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