NONLYMPHOBLASTIC LYMPHOMA IN CHILDREN - HISTOLOGY AND STAGE-RELATED RESPONSE TO THERAPY - A PEDIATRIC-ONCOLOGY-GROUP STUDY

From May 1979 to March 1983, 93 eligible patients with nonlymphoblastic lymphoma (NLBL) were treated by members of the Pediatric Oncology Group (POG) with Adriamycin (doxorubicin; Adria Laboratories, Columbus, OH), vincristine, prednisone, cyclophosphamide, and mercaptopurine (ACOP+); CNS prophylaxis with intrathecal (IT) methotrexate, hydrocortisone, and cranial irradiation (2,400 rads), and radiation therapy to the primary disease were administered in stages I and II, and to residual disease in stages III and IV. Duration of treatment was 2 years for stages I, II, and III and 3 years for stage IV disease. Of the 93 patients entered onto the study, 47 had diffuse small noncleaved-cell lymphoma (DSNCL), 38 had diffuse large-cell lymphoma (DLCL), and eight had other histologies. Localized disease (stages I and II) was present in 51 patients, and 42 had advanced (stages III and IV) disease. The study confirmed previously reported importance of stage with a 4-year event-free survival (EPS) of 78% (SE ± 7%) for patients with localized disease as compared with 44% (SE ± 9%) in patients with advanced disease (P ≤ .001). In localized disease, seven of 11 adverse events occurred in patients who were off therapy and more than 30 months after the initial diagnosis (relapse, three; second malignancy, two; death in remission, two). Large-cell histology proved to be an important prognostic factor in patients with stages III and IV disease with EFS at 4 years of 67% (SE ± 11%) for DLCL versus 17% (SE ± 11%) for DSNCL (P ≤ .001). We conclude that it is important to distinguish histologically between small noncleaved-cell and large-cell types of NLBL as a basis for further controlled clinical trials. © 1991 by American Society of Clinical Oncology.