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.

Additional Metadata
Keywords complete resection, marginal zone lymphoma, outcome, watch-and-wait
Persistent URL dx.doi.org/10.1002/pbc.26932, hdl.handle.net/1765/112604
Journal Pediatric Blood & Cancer
Citation
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