Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group
Aleksandra Wieczorek,
Joanna Stefanowicz,
Marcin Hennig,
Elzbieta Adamkiewicz-Drozynska,
Marzena Stypinska,
Bozenna Dembowska-Baginska,
Zuzanna Gamrot,
Mariola Woszczyk,
Julia Geisler,
Tomasz Szczepanski,
Szymon Skoczen,
Marek Ussowicz,
Monika Pogorzala,
Szymon Janczar,
Walentyna Balwierz
Affiliations
Aleksandra Wieczorek
Department of Paediatric Oncology and Haematology, Faculty of Medicine, Jagiellonian University, Medical College
Joanna Stefanowicz
Department of Paediatrics, Haematology and Oncology, Medical University of Gdansk
Marcin Hennig
Department of Paediatrics, Haematology and Oncology, Medical University of Gdansk
Elzbieta Adamkiewicz-Drozynska
Department of Paediatrics, Haematology and Oncology, Medical University of Gdansk
Marzena Stypinska
Department of Oncology, The Children Memorial Health Institute in Warsaw
Bozenna Dembowska-Baginska
Department of Oncology, The Children Memorial Health Institute in Warsaw
Zuzanna Gamrot
Unit of Paediatric Haematology and Oncology, City Hospital
Mariola Woszczyk
Unit of Paediatric Haematology and Oncology, City Hospital
Julia Geisler
Department of Paediatric Haematology and Oncology, Medical University of Silesia
Tomasz Szczepanski
Department of Paediatric Haematology and Oncology, Medical University of Silesia
Szymon Skoczen
Department of Paediatric Oncology and Haematology, Faculty of Medicine, Jagiellonian University, Medical College
Marek Ussowicz
Department and Clinic of Paediatric Oncology, Haematology and Bone Marrow Transplantation, Wroclaw Medical University
Monika Pogorzala
Paediatric Haematology and Oncology, Collegium Medicum, Nicolaus Copernicus University
Szymon Janczar
Department of Paediatrics, Oncology and Haematology, Medical University of Lodz
Walentyna Balwierz
Department of Paediatric Oncology and Haematology, Faculty of Medicine, Jagiellonian University, Medical College
Abstract Although isolated central nervous system (CNS) relapses are rare, they may become a serious clinical problem in intensively treated patients with high-risk neuroblastoma (NBL). The aim of this study is the presentation and assessment of the incidence and clinical course of isolated CNS relapses. Retrospective analysis involved 848 NBL patients treated from 2001 to 2019 at 8 centres of the Polish Paediatric Solid Tumours Study Group (PPSTSG). Group characteristics at diagnosis, treatment and patterns of relapse were analysed. Observation was completed in December 2020. We analysed 286 high risk patients, including 16 infants. Isolated CNS relapse, defined as the presence of a tumour in brain parenchyma or leptomeningeal involvement, was found in 13 patients (4.5%; 8.4% of all relapses), all of whom were stage 4 at diagnosis. Isolated CNS relapses seem to be more common in young patients with stage 4 MYCN amplified NBL, and in this group they may occur early during first line therapy. The only or the first symptom may be bleeding into the CNS, especially in younger children, even without a clear relapse picture on imaging, or the relapse may be clinically asymptomatic and found during routine screening. Although the incidence of isolated CNS relapses is not statistically significantly higher in patients after immunotherapy, their occurrence should be carefully monitored, especially in intensively treated infants, with potential disruption of the brain-blood barrier.
