Children and adolescents with follicular lymphoma have an excellent prognosis with either limited chemotherapy or with a 'watch and wait' strategy after complete resection

Abstract

PURPOSE/OBJECTIVE: Data on clinical features and outcome in pediatric follicular lymphoma (pFL) are scarce. The aim of this retrospective study including 13 EICNHL (European Intergroup for Childhood non-Hodgkin lymphoma) and/or i-BFM (Berlin-Frankfurt-Munster) Study Group members was to assess clinical characteristics and course in a series of 63 pFL patients. MATERIALS AND METHODS: Between May and December 2011, we performed an international survey of pFL including only patients with nationally centrally reviewed histopathology. The survey included questions on demographics [age, gender, sites of involvement, stage of disease, pre-therapeutic lactate dehydrogenase (LDH) level], treatment (surgery, chemotherapy, radiotherapy) and outcome (date of remission, relapse, death, last follow-up). RESULTS: Pediatric FL was found to be associated with male gender (3:1), older age (72% ≥ 10 years old), low serum LDH levels (<500 U/l in 75%), grade 3 histology (in 88%), and limited disease (87% stage I/II disease) mostly involving the peripheral lymph nodes. Forty-four/63 patients received any polychemotherapy and one/63 rituximab only while 17/63 underwent a ‘watch and wait’ strategy. Of 36 stage I patients 30 had complete resections. Only one patient relapsed; 2-year event-free survival and overall survival were 94% +/- 5% and 100%, respectively, after a median follow-up of 2.2 years. CONCLUSIONS: As children with non-resectable pFL had an excellent outcome with multidrug chemotherapy which is associated with acute- and long-term toxicity, multinational controlled trials have to be performed, taking genetics into account, to establish not only that no chemotherapy is a safe approach in stage I patients with complete resection, but low-intensity chemotherapy +/- monoclonal antibodies is sufficient for patients with non-completely resectable disease.