Malignant non-Hodgkin's lymphoma (NHL) in childhood. Retrospective analysis of 34 cases.

Of 47 children with an initial diagnosis of lymphosarcoma, reticulosarcoma or Non-Hodgkin's lymphoma (NHL), 13 had to be excluded at the histologic reevaluation: in 10 an undifferentiated sarcoma, in 2 Hodgkin lymphoma was found; in one patient no definite classification of the tumor was possible. Of the remaining 34 patients there were 26 boys and 8 girls. One patient had a nodular, 33 a diffuse NHL. Of the latter 16 had a Burkitt-type (LB-), 3 a lymphoblastic, convoluted (LC-), 8 a lymphoblastic, "other" (LO-) and 6 a histiocytoid (H-) NHL. Primary localization: abdomen: 13/34; "peripheral" lymph nodes: 9/34; mediastinum: 5/34; nasopharynx: 4/34; subcutis: 2/34; skeleton: 1/34. Twelve of 17 NHL with primary localization in the abdomen or nasopharynx were LB-NHL, 8/14 NHL with primary localization in "peripheral" nodes or mediastinum were LC- or LO-NHL. Only 2/17 NHL with abdominal or nasopharyngeal primary, but 9/14 NHL with "peripheral" nodal or mediastinal primary developed leukemic extension and/or CNS involvement. 6 of 34 patients are living without evidence of disease for 1 1/2+ to 13+ years; 5/34 died but lived for 85, 57, 37, 22 and 22 months; 9/34 lived 6--12 months; 14/34 died within less than 6 months. Patients with abdominal primary either died within 5 months or survived (for 165+, 63+ and 25+ months). Aggressive local therapy (surgery and radiotherapy with approximately 4000 R) may be adequate for strictly localized (stage I) disease, particularly if the primary localization is abdominal. In all other diffuse NHL of childhood an early, aggressive chemotherapy, later combined with radiotherapy to bulk disease and prophylactic CNS-treatment is essential for inducing long-term remissions and, possibly, cures. For prognosis the primary localization appeared to be more important than histology and stage. The most decisive factor, however, is therapy.