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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

par 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
Référence Pediatric blood & cancer, 65, 4, e26932
Publication Publié, 2018-04

Article révisé par les pairs

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Résumé :

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.