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

Pediatr Blood Cancer. 2018 Apr;65(4). doi: 10.1002/pbc.26932. Epub 2017 Dec 29.

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 and observation only.

Keywords: complete resection; marginal zone lymphoma; outcome; watch-and-wait.

Publication types

  • Clinical Trial
  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease-Free Survival
  • Female
  • Follow-Up Studies
  • Humans
  • Lymphoma, B-Cell, Marginal Zone / mortality*
  • Lymphoma, B-Cell, Marginal Zone / physiopathology*
  • Lymphoma, B-Cell, Marginal Zone / therapy*
  • Male
  • Retrospective Studies
  • Survival Rate