Open questions and evidence on new therapeutic standards for limited-stage diffuse large B-cell lymphomas

Abstract

Diffuse large B-cell lymphoma (DLBCL) is the most common non-Hodgkin lymphoma, with limited-stage defined as stage I or II disease, having around 25-30% of newly diagnosed DLBCL this presentation. Risk stratification, initial treatment options, and relapse patterns are distinct from advanced-stage disease, but there is limited data on the impact of biologic features on outcome. Patients presenting with localized disease have excellent outcomes with an estimated survival at two years around 90%. Over the past years several studies have attempted to evaluate the optimal number of chemotherapy cycles, the role of radiotherapy and PET- adapted approaches in order to maximize success of therapy and minimize toxicity. Special consideration must still be given to cases of bulky disease, extranodal disease, fully resected scenarios and adverse biologic features such as high-grade B-cell lymphoma with double/triple hit rearrangements. In this review the new therapeutic options based on the best available medical evidence, as well as the remaining open questions will be discussed.