NON-HODGKINS-LYMPHOMAS OF CHILDHOOD AND ADOLESCENCE - RESULTS OF A TREATMENT STRATIFIED FOR BIOLOGIC SUBTYPES AND STAGE - A REPORT OF THE BERLIN-FRANKFURT-MUNSTER GROUP

Purpose: To prove the efficacy of a treatment stratified according to histology for children with non-Hodgkin's lymphoma (NHL), including ac ute B-cell leukemia (B-ALL). Patients and Methods: From October 1986 t o March 1990, 302 assessable patients, 0.6 to 17.8 years of age, with newly diagnosed NHL were enrolled onto study ALL/NHL-BFM 86. Fifty per cent of patients had Burkitt-type lymphomas, including B-ALL; 24% had lymphoblastic lymphoma; 18% had diffuse large-cell lymphoma; and 8% he rd an NHL not further classified. Therapy group B included Burkitt's-t ype lymphomas, B-ALL, and most large-cell lymphomas including Ki-1 ana plastic large-cell lymphoma. Patients with stage I and II disease rese cted received three, while all others received six, 5-day therapy cour ses (dexamethasone, methotrexate [MTX] 0.5 g/m(2) [5 g/m(2) for stage IV and B-ALL], and intrathecal [IT] therapy in each course, plus ifosf amide, cytarabine, and etoposide alternating with cyclophosphamide and doxorubicin). Therapy for group non-B patients (lymphoblastic lymphom a and pleomorphic T-cell lymphoma [PTCL]) consisted of a Berlin-Frankf urt-Munster (BFM) acute lymphoblastic leukemia protocol, including cra nial irradiation for advanced stage. Local therapy was restricted to p atients with incomplete tumor regression. Results: The probabilities o f event-free survival (pEFS) at 7 years were 80% +/- 2% for the whole group, 81% +/- 3% for group B (n = 225), and 78% +/- 5% for group non- B (n = 77) with ct follow-up duration of 3.6 to 7 years (median 5 year s). Treatment results were comparable between NHL subtypes, except for PTCL, in which three of four patients suffered from relapse. Local di sease manifestations were the most frequent site of failure. Conclusio n: This therapy strategy provided patients of all NHL subtypes with on equally high chance to survive event-free, except patients with PTCL. With reduced systemic failure, local tumor control may become more im portant.