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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Keywords complete resection, marginal zone lymphoma, outcome, watch-and-wait
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Journal Pediatric Blood & Cancer
Ronceray, L. (Leila), Abla, O, Barzilai-Birenboim, S. (Shlomit), Bomken, S, Chiang, A.K.S. (Alan K. S.), Jazbec, J, … Attarbaschi, A. (2018). Children and adolescents with marginal zone lymphoma have an excellent prognosis with limited chemotherapy or a watch-and-wait strategy after complete resection. Pediatric Blood & Cancer, 65(4). doi:10.1002/pbc.26932