Djurisic, Marina (12769932200)Marina (12769932200)DjurisicSarajlija, Adrijan (26027638400)Adrijan (26027638400)SarajlijaRadivojevic, Danijela (12769357500)Danijela (12769357500)RadivojevicCirkovic, Sanja (56627166200)Sanja (56627166200)CirkovicDjokic, Dragoljub (36619461900)Dragoljub (36619461900)DjokicDjuricic, Slavisa (6603108728)Slavisa (6603108728)DjuricicSamardzija, Gordana (56177152500)Gordana (56177152500)SamardzijaPasic, Srdjan (55904557400)Srdjan (55904557400)Pasic2025-06-122025-06-122025https://doi.org/10.1097/MPH.0000000000002965https://www.scopus.com/inward/record.uri?eid=2-s2.0-85210306734&doi=10.1097%2fMPH.0000000000002965&partnerID=40&md5=9473206e1d8acd2f249bd40850c29216https://remedy.med.bg.ac.rs/handle/123456789/658Nijmegen breakage syndrome (NBS) is a rare primary immunodeficiency disease due to a pathogenic variant in the NBN gene causing impaired DNA repair and increased predisposition for lymphoid malignancy. By contrast, solid tumors have been rarely reported. Neuroblastoma (NB) is a rare childhood solid tumor, associated with the worse outcome if MYCN oncogene is amplified. We describe 2 young pediatric patients with NBS who developed high-risk NB. The first patient died shortly after chemotherapy was introduced. The second patient successfully received modified chemotherapy resulting in clinical remission lasting 2 years after an initial diagnosis of NB. © 2024 Wolters Kluwer Health, Inc. All rights reserved.DNA damage responseMYCN amplificationNBN geneneuroblastomaNijmegen breakage syndrome