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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.

Journal article
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
Published in Pediatric blood & cancer
Volume 65
Issue 4
Pages e26932
ISSN 1545-5017
Publication year 2018
Published at Institute of Clinical Sciences, Department of Pediatrics
Pages e26932
Language en
Links dx.doi.org/10.1002/pbc.26932
www.ncbi.nlm.nih.gov/entrez/query.f...
Keywords complete resection; marginal zone lymphoma; outcome; watch-and-wait
Subject categories Pediatrics

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.

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