Improving Risk Stratification to Guide Treatment Decisions for Children, Adolescents, and Young Adults With Hodgkin Lymphoma
Authors: Lianna J. Marks
Journal: Journal of the National Comprehensive Cancer Network (2021)

odgkin lymphoma (HL) is a highly curable disease with multiple evidence- based treatment options for children and adolescents.With the expectation of long-term survival of patients with HL comes the responsibility to use treatment regimens that reduce the risk of late effects, such as cardiovascular disease, infertility, pulmonary complications, and second malignant neoplasm (SMN).Recognizing the unique needs of this patient population, clinical trials developed for pediatric and adolescent patients embrace risk-adapted, response-based protocols focused on minimizing long-term toxicity while preserving high cure rates.The preferred primary treatment regimens recommended in the new NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Pediatric Hodgkin Lymphoma, including the Children's Oncology Group (COG) studies AHOD0031 and AHOD1331 and the European Pediatric and Adolescent Hodgkin Lymphoma Network (EuroNet-HD) study EuroNet-PHL-C1, build on previous studies developed with these goals in mind.For adolescents and young adults (AYAs) ages 15 to 39 years, who make up the majority of patients with HL, an important initial decision is whether to treat on pediatric or adult protocols.In general, the recommended approaches consist of several cycles (2-6) of multiagent chemotherapy with or without involved-site radiation therapy (ISRT), depending on initial risk group and interim PET response.Although enrollment on a phase III clinical trial is encouraged if available, choosing the best treatment approach for an individual patient can be a complex choice given the different treatment options and desire to balance individual risk factors and treatment toxicities.Initial staging of patients with HL is the foundation for developing a riskadapted treatment plan.Both pediatric and adult groups rely on the Ann Arbor classification system for staging, but treatment approaches, risk stratification, and response criteria have evolved in parallel due to differences in clinical practice between disciplines.Following consensus efforts by adult lymphoma cooperative groups, the international Staging Evaluation and Response Criteria Harmonization (SEARCH) for Childhood, Adolescent and Young Adult Hodgkin Lymphoma (CAYAHL) group was formed to standardize the staging process and allow for improved comparisons of pediatric cooperative group trials. 1 However, comparing outcomes between trials is further complicated by the differing risk stratification and group allocation approaches used not only by adult and pediatric cooperative group trials but also between pediatric HL groups and between generations of trials within the same group.The criteria for unfavorable clinical presentations used to determine risk stratification vary among protocols, but typically include stage, the presence of "B" symptoms, bulky lymphadenopathy, and extranodal extension to contiguous structures (Elesions).Of note, adult cooperative groups and the EuroNet-HD pediatric