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

Abstract

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. Pediatr Blood Cancer. 2010;56:70–76.