Clinical characteristics and outcome of pediatric and adolescent patients with follicular lymphoma - The EICNHL and i-BFM study groups experience

Abstract

BACKGROUND: Follicular lymphoma (FL) rarely occurs in children and adolescents accounting for 2% of all B-cell non-Hodgkin’s lymphomas. Moreover, comprehensive data on clinical, histopathologic and outcome characteristics are scarce. OBJECTIVES/METHODS: The aim of this multinational retrospective study including 13 EICNHL and/or i-BFM study group members was to assess these parameters in a series of 63 patients with FL. RESULTS: The male to female ratio was 3:1 and median age was 13 years. Thirty-six suffered from stage I, 19 from stage II, six from stage III and two from stage IV disease. Median serum LDH level was 252 U/l. Ten percent demonstrated grade I/II and 66% grade III histopathology (not available in 24%). Seventy-nine percent had peripheral lymph node involvement. Of 36 stage I patients 30 had a complete resection. Of all 63 patients 44 received any chemotherapy and one rituximab only while 17 underwent a watch and wait strategy. Only one patient relapsed; 4-year pEFS was 94 ± 5%, overall survival 100% after a median follow-up of 2.2 years. CONCLUSIONS: FL in children and adolescents seems to be associated with male gender, >10 years of age, limited stage of disease, low LDH levels and peripheral lymph node involvement. Treatment outcome is excellent.