Malignant lymphomas in children and adolescents.

Abstract

Malignant lymphomas constitute the third most common group of childhood cancers in the United States. Hodgkin's disease (HD) exhibits a bimodal age peak, with the first peak occurring in the 15-34-year age group. Nodular sclerosis is the most common histologic subtype of HD seen in children. The disease tends to be primarily supradiaphragmatic at presentation. Accurate staging is essential for determination of appropriate therapy. Staging laparotomy with splenectomy is indicated for those patients with clinically localized disease whose therapy may be altered by pathologic staging. Radiation therapy is curative in the great majority of patients with localized disease. Multimodality therapy has been employed with increasing success in patients with advanced disease. The late sequelae of therapy must be considered in the design of optimum therapeutic regimens. More than 90% of non-Hodgkin's lymphoma (NHL) in childhood can be grouped into one of three histologic subtypes: lymphoblastic, undifferentiated, and diffuse large cell type. Lymphoblastic lymphomas most commonly present with mediastinal involvement. The majority of nonlymphoblastic lymphomas arise within the abdomen. Because of the tendency of NHL for extralymphatic dissemination, systemic therapy is always required. Treatment is determined by stage and histology. Significant improvements in survival rates have resulted from the development of effective combination chemotherapy programs. Cure rates in excess of 90% can be expected in children with localized disease. The outlook for children with advanced disease of undifferentiated histology remains poor. The presence of central nervous system involvement at diagnosis is a particularly ominous sign.