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Malignant lymphomas in children and adolescents
1Department of Pediatrics, Roswell Park Memorial Institute, Buffalo, NY 14263.
Insights
Childhood lymphomas, including Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL), require accurate staging and tailored therapy. Advances in chemotherapy and radiation offer high cure rates for localized disease, but advanced or disseminated NHL poses challenges.
Area of Science:
- Pediatric Oncology
- Hematology
- Cancer Biology
Background:
- Malignant lymphomas are a significant cause of childhood cancer in the U.S.
- Hodgkin's disease (HD) has a bimodal age distribution, with nodular sclerosis being the most common subtype in children, often presenting supradiaphragmatically.
- Childhood non-Hodgkin's lymphoma (NHL) comprises lymphoblastic, undifferentiated, and diffuse large cell types, frequently presenting with mediastinal or abdominal involvement and extralymphatic dissemination.
Purpose of the Study:
- To review the epidemiology, staging, and therapeutic approaches for childhood Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL).
- To highlight the importance of accurate staging for treatment selection in pediatric lymphomas.
- To discuss treatment modalities, including radiation therapy, chemotherapy, and multimodality approaches, and their impact on survival and late sequelae.
Main Methods:
- Review of epidemiological data and established treatment protocols for pediatric HD and NHL.
- Analysis of diagnostic criteria, staging systems, and therapeutic outcomes.
- Consideration of prognostic factors, including histology, stage, and central nervous system involvement.
Main Results:
- Hodgkin's disease (HD) in children is often localized and curable with radiation therapy; advanced stages benefit from multimodality therapy.
- Localized childhood non-Hodgkin's lymphoma (NHL) has high cure rates (>90%) with combination chemotherapy.
- Advanced or undifferentiated NHL, especially with central nervous system involvement, has a poorer prognosis despite therapeutic advances.
Conclusions:
- Accurate staging and histology-specific treatment are crucial for optimizing outcomes in pediatric lymphomas.
- Multimodality therapy and effective chemotherapy regimens have significantly improved survival rates for childhood HD and NHL.
- Long-term sequelae of therapy necessitate careful consideration in treatment planning for pediatric cancer survivors.
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.