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Children and adolescents with marginal zone lymphoma have an excellent prognosis with limited chemotherapy or a watch‐and‐wait strategy after complete resection
Authors:Leila Ronceray  Oussama Abla  Shlomit Barzilai‐Birenboim  Simon Bomken  Alan KS Chiang  Janez Jazbec  Edita Kabickova  Jelena Lazic  Auke Beishuizen  Karin Mellgren  Fumiko Tanaka  Marta Pillon  Christine Devalck  Marina Gouttenoire  Olga Makarova  Birgit Burkhardt  Andishe Attarbaschi
Institution:1. Pediatric Hematology and Oncology, St. Anna Children's Hospital, Medical University of Vienna, Vienna, Austria;2. Division of Hematology and Oncology, Department of Pediatrics, Hospital for Sick Children, Toronto, Canada;3. Pediatric Hematology and Oncology, Schneider Children's Medical Center of Israel, Petah‐Tivka, Israel;4. Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel;5. Northern Institute for Cancer Research, Newcastle University, Newcastle, UK;6. Department of Pediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, Queen Mary Hospital, The University of Hong Kong, Pokfulam, Hong Kong;7. Division of Pediatrics, Hematology and Oncology, University Medical Center Ljubljana, Ljubljana, Slovenia;8. Pediatric Hematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic;9. Pediatric Hematology and Oncology, University Children's Hospital, School of Medicine University of Belgrade, Belgrade, Serbia;10. Pediatric Hematology and Oncology, Erasmus MC‐Sophia Children's Hospital, Rotterdam, The Netherlands;11. Pediatric Hematology and Oncology, The Queen Silvia's Hospital for Children and Adolescents, University of G?teborg, G?teborg, Sweden;12. Department of Pediatrics, Saiseikai Yokohamashi Nanbu Hospital, Yokohama, Japan;13. Pediatric Hematology and Oncology, University of Padova, Padova, Italy;14. Pediatric Hematology and Oncology, Hopital Universitaire des Enfants Reine Fabiola, Brussels, Belgium;15. Pediatric Hematology and Oncology, University Hospital, Saint Etienne, France;16. Pediatric Hematology and Oncology, University of Münster, Münster, Germany
Abstract:Data on management of pediatric marginal zone lymphoma (MZL) are scarce. This retrospective study assessed characteristics and outcome in 66 patients who were <18 years old. Forty‐four (67%) had an extranodal MZL (EMZL), 21 (32%) a nodal MZL (NMZL), and one patient a splenic MZL. Thirty‐three patients (50%) received a variable combination of adjuvant chemotherapy/immunotherapy/radiotherapy, while the remainder, including 20 of 21 with NMZL, entered an active observation period. Overall survival was excellent (98 ± 2%), although 11 patients relapsed (17%; NMZL, n = 1; EMZL, n = 10), seven after any therapy and four after complete resection only. In conclusion, outcome of NZML, in particular, seems to be excellent after (in)complete resection and observation only.
Keywords:complete resection  marginal zone lymphoma  outcome  watch‐and‐wait
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