Children and adolescents with marginal zone lymphoma have an excellent prognosis with limited chemotherapy or a watch-and-wait strategy after complete resection.
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.
['Ronceray|Leila|L|', 'Abla|Oussama|O|', 'Barzilai-Birenboim|Shlomit|S|', 'Bomken|Simon|S|', 'Chiang|Alan Ks|AK|', 'Jazbec|Janez|J|', 'Kabickova|Edita|E|', 'Lazic|Jelena|J|', 'Beishuizen|Auke|A|', 'Mellgren|Karin|K|', 'Tanaka|Fumiko|F|', 'Pillon|Marta|M|0000-0002-1190-3198', 'Devalck|Christine|C|', 'Gouttenoire|Marina|M|', 'Makarova|Olga|O|', 'Burkhardt|Birgit|B|', 'Attarbaschi|Andishe|A|0000-0002-9285-6898', '|||']
D000293:Adolescent D002648:Child D002675:Child, Preschool D018572:Disease-Free Survival D005260:Female D005500:Follow-Up Studies D006801:Humans D018442:Lymphoma, B-Cell, Marginal Zone D008297:Male D012189:Retrospective Studies D015996:Survival Rate
marginal zone lymphoma watch-and-wait complete resection outcome