Cyclophosphamide monotherapy in children with Burkitt lymphoma: A study from the French-African Pediatric Oncology Group (GFAOP)

Background: The French African Group of Pediatric Oncology was set-up to improve quality of care for children with cancer. Preliminary observations on the efficacy in Burkitt lymphoma (BL) of a cyclophosphamide monotherapy (CPM) have been published. We report the results of a multicentric prospective study combining first-line CPM and a multidrug second-line chemotherapy (SC) for refractory/relapsed patients. Procedure: Patients ≤18 years with Burkitt or Burkitt-like lymphoma, were included in six countries (Burkina-Faso, Cameroon, Ivory Coast, Madagascar, Mali, and Senegal). All patients received three weekly CPM courses (1.2 g/m2 IV with intrathecal methotrexate and hydrocortisone), stage 3/4 patients received three further courses. SC added methotrexate, vincristine, cytarabine, and prednisone. Results: There were 178 patients included (42 stage 1/2, 134 stage 3/4, and 2 unknown). Isolated facial localization was found in 41 patients, diffuse abdominal involvement in 120 patients including 65 with both. Nine early deaths were reported, toxicity occurred in 136/743 courses (83 patients) and was predominantly hematological. After CPM, complete remission (CR) rate was 47% with a 33% EFS. Because of rapid progression 76/108 eligible patients (85 primary refractory and 23 relapses) received SC resulting in 35.7% CR but a 21% toxic death rate. The OS of the whole strategy was 50.5% and correlated to stage. Conclusion: A prospective multicentric study on BL was feasible in very low-income countries. CPM can be recommended in stage 1-2 because of optimal cost/benefit ratio. However, more intensive strategies, still adapted to socio-economic conditions, are required for advanced stages 3 and 4. © 2010 Wiley-Liss, Inc.