PRIMARY PERIPHERAL NODAL LYMPHOMA IN CHILDREN

Background. In this series of 208 pediatric patients with non-Hodgkin (NHL) studied from 1971 to 1986, 84 patients (40.4%) had nodal lymphom as; 40 (19.2%) of these patients had peripheral nodal lymphoma and 44 (21.2%) had mediastinal lymphoma. Methods. Forty pediatric patients wi th primary peripheral nodal lymphoma were treated at Memorial Sloan-Ke ttering Cancer Center with the LSA2-L2 protocol from July 1971 to Janu ary 1986. Informed consent was obtained from all patients and/or guard ians. Results. There were 26 male patients and 14 female patients, wit h a median age of 10 years. Two patients had Stage I disease, 5 Stage II, 9 Stage III, 8 Stage IVA (< 25% blasts in the bone marrow), and 16 Stage IVB (> 25% blasts in the bone marrow). The last patient with St age IVB disease was entered in 1977, a time when the philosophy of tre atment for leukemia-lymphomas had not yet evolved completely. Most of these lymphomas were high-grade lymphoblastic lymphomas, followed by i mmunoblastic lymphomas and reticulosarcomas. The event-free survival r ate of this group of patients was 75%, with all patients having comple ted therapy, and a median observation time of more than 10 years witho ut therapy. The lymphoma-free survival rate was 80%. Sex, age, and sta ge were not of prognostic significance. There was no significant diffe rence in survival between patients with lymphoblastic and histiocytic lymphomas (75% versus 64%, respectively). There was no significant dif ference in survival between the patients with high-grade and medium-gr ade lymphomas (75% versus 78%, respectively). Lactic dehydrogenase (LD H) in this primary site was not indicative of extent or bulk of diseas e and did not affect survival negatively. Radiation therapy and dose i ntensity of chemotherapy influenced survival by promoting rapid and mo re complete cell kill, helping prevent the emergence of resistant cell s. Conclusions. Although primary peripheral nodal lymphoma usually is disseminated at diagnosis, it is still a highly curable disease when t reated aggressively. The lymphoma-free survival rate for patients with primary nodal NHL with marrow involvement is 75%, and this subsequent ly has led to a different philosophy in the treatment of high-risk leu kemias and lymphoma-leukemias with the NY-I and NY-II protocols, with excellent results.