Children and adolescents with marginal zone lymphoma have an excellent prognosis with limited chemotherapy or a watch-and-wait strategy after complete resection

Ronceray, Leila
Mellgren, Karin
Tanaka, Fumiko
Pillon, Marta
Devalck, Christine
Gouttenoire, Marina
Makarova, Olga
Burkhardt, Birgit
Attarbaschi, Andishe
Abla, Oussama
Barzilai-Birenboim, Shlomit
Bomken, Simon
Chiang, Alan Kwok Shing
Jazbec, Janez
Kabickova, Edita
Lazic, Jelena
Beishuizen, Auke

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Publication date

April 2018

DOI

10.1002/pbc.26932

Publisher

Wiley

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...