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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 |
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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 |
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.