Primary mediastinal B-cell lymphoma in the pediatric patient: Can a rational approach to therapy be based on adult studies?

The literature on adult and pediatric primary mediastinal B-cell lymphoma (PMBCL) was reviewed and compared. Biologically, adult PMBCL has more similarities to Hodgkin Lymphoma (HL) than diffuse large B-cell lymphoma (DLBCL). Pediatric studies suggest that the biology is similar to that in adults. Median age of children is 14.3 years and the overall survival (OS) is reported as 78.6% and event-free survival (EFS) as 67.4%. Adverse prognostic factors included LDH >500 and mass size over 10 cm, with a trend towards better survival in younger patients. Studies in adults show better survival with intensified chemotherapy and the addition of rituximab. Data on the use of radiation therapy show improved CR rates and survival with addition of involved field radiation therapy (IFRT). Positron emission tomography (PET) with computerized tomography (CT) imaging response-assessment after two courses and at therapy-end may allow for the rational use of IFRT in pediatric/adolescent patients who are more susceptible to development of adverse late effects. (c) 2008 Wiley-Liss, Inc.