CNS involvement in children with newly diagnosed non-Hodgkin's lymphoma

Purpose: To determine the frequency of CNS involvement at diagnosis of non- Hodgkin's lymphoma (NHL), to characterize its pattern of presentation, and to determine its prognostic significance. Patients and Methods: We reviewed the records of 445 children (1975 through 1995) diagnosed with NHL (small noncleaved cell NHL/B-cell acute lymphobla stic leukemia [SNCC NHL/B-ALL], 201 patients; lymphoblastic, 113; large cel l, 119; other, 12). Tumor burden wets estimated by serum lactate dehydrogen ase (LDH) measurement and reclassification of disease stage irrespective of CNS involvement (modified stage). Results: Thirty-six of 445 children with newly diagnosed NHL had CNS involv ement (lymphoma cells in the CSF [n = 23], cranial nerve palsy [n = 9], bot h features [n = 4]), representing 13%, 7%, and 1% of small noncleaved cell lymphoma, lymphoblastic lymphoma, and large-cell cases, respectively. By un ivariate analysis, CNS disease at diagnosis did not significantly impact ev ent-free survival (P = .095), whereas stage and LDH did; however, children with CNS disease at diagnosis were at 2.0 times greater risk of death than those without CNS disease at diagnosis. In a multivariate analysis, CNS dis ease was not significantly associated with either overall or event-free sur vival, whereas both serum LDH and stage influenced both overall and event-f ree survival. Among cases of SNCC NHL/B-ALL, CNS disease was significantly associated with event-free and overall survival (univariate analysis); howe ver, in multivariate analysis, only LDH had independent prognostic signific ance. Elevated serum LDH or higher modified stage were associated with a tr end toward poorer overall survival among children with CNS disease. Conclusion: A greater tumor burden at diagnosis adversely influences the tr eatment outcome of children with NHL and CNS disease at diagnosis, suggesti ng a need for ongoing improvement in both systemic and CNS-directed therapy . (C) 2000 by American Society of Clinical Oncology.