Abstract
BACKGROUND/AIM: Non-B non-Hodgkin lymphomas (NHL) represent over 30 T/NK lymphoma types. The majority of them are T-cell lymphoblastic lymphomas (TLL) and anaplastic large cell lymphomas (ALCL). Other rare non-B NHLs represent a diverse group of neoplasms, usually excluded from clinical trials. This study analyzed outcomes in pediatric patients with non-B NHL in a single oncology center with particular emphasis on patients with rare NHLs. PATIENTS AND METHODS: We retrospectively analyzed data from patients <18 years with newly diagnosed non-B NHL treated at the Department of Pediatric Hematology and Oncology in Bydgoszcz between 2002 and 2022. The probability of 5-year overall survival (pOS) and event-free survival (pEFS) were calculated for the entire cohort and patients with TLL and ALCL. The clinical course for patients with rare non-B NHL was described in detail. RESULTS: Twenty-six children were eligible for analysis. Fourteen patients were diagnosed with ALCL, nine with TLL, and three with rare NHL types (subcutaneous panniculitis-like T-cell lymphoma, extranodal NK/T-cell lymphoma and hydroa vacciniforme-like lymphoproliferative disease associated lymphoma). For the entire group, the 5-year pOS was 83.7% and the 5-year pEFS was 72.4%. For TLL and ALCL, the outcomes were comparable with those achieved in clinical trials. Patients with rare NHL were treated according to individualized therapy recommendations based on physicians' expertise and available case report descriptions. CONCLUSION: There is a lack of knowledge on optimal therapeutic strategies for rare NHLs. It is crucial to create trials dedicated to uncommon NHLs and establish therapy guidelines for these patients.