Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group
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 rela...
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| Format: | Article |
| Language: | English |
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BMC
2022-06-01
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| Series: | BMC Cancer |
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| Online Access: | https://doi.org/10.1186/s12885-022-09776-x |
| _version_ | 1818248599290511360 |
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| author | 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 |
| author_facet | 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 |
| author_sort | Aleksandra Wieczorek |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-12T15:23:09Z |
| format | Article |
| id | doaj.art-7aad612c386440b495ee93ae95b9c5ff |
| institution | Directory Open Access Journal |
| issn | 1471-2407 |
| language | English |
| last_indexed | 2024-12-12T15:23:09Z |
| publishDate | 2022-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Cancer |
| spelling | doaj.art-7aad612c386440b495ee93ae95b9c5ff2022-12-22T00:20:19ZengBMCBMC Cancer1471-24072022-06-012211910.1186/s12885-022-09776-xIsolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study GroupAleksandra Wieczorek0Joanna Stefanowicz1Marcin Hennig2Elzbieta Adamkiewicz-Drozynska3Marzena Stypinska4Bozenna Dembowska-Baginska5Zuzanna Gamrot6Mariola Woszczyk7Julia Geisler8Tomasz Szczepanski9Szymon Skoczen10Marek Ussowicz11Monika Pogorzala12Szymon Janczar13Walentyna Balwierz14Department of Paediatric Oncology and Haematology, Faculty of Medicine, Jagiellonian University, Medical CollegeDepartment of Paediatrics, Haematology and Oncology, Medical University of GdanskDepartment of Paediatrics, Haematology and Oncology, Medical University of GdanskDepartment of Paediatrics, Haematology and Oncology, Medical University of GdanskDepartment of Oncology, The Children Memorial Health Institute in WarsawDepartment of Oncology, The Children Memorial Health Institute in WarsawUnit of Paediatric Haematology and Oncology, City HospitalUnit of Paediatric Haematology and Oncology, City HospitalDepartment of Paediatric Haematology and Oncology, Medical University of SilesiaDepartment of Paediatric Haematology and Oncology, Medical University of SilesiaDepartment of Paediatric Oncology and Haematology, Faculty of Medicine, Jagiellonian University, Medical CollegeDepartment and Clinic of Paediatric Oncology, Haematology and Bone Marrow Transplantation, Wroclaw Medical UniversityPaediatric Haematology and Oncology, Collegium Medicum, Nicolaus Copernicus UniversityDepartment of Paediatrics, Oncology and Haematology, Medical University of LodzDepartment of Paediatric Oncology and Haematology, Faculty of Medicine, Jagiellonian University, Medical CollegeAbstract 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.https://doi.org/10.1186/s12885-022-09776-xCentral nervous systemClinical courseIsolated relapseMetastasesNeuroblastoma |
| spellingShingle | 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 Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group BMC Cancer Central nervous system Clinical course Isolated relapse Metastases Neuroblastoma |
| title | Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group |
| title_full | Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group |
| title_fullStr | Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group |
| title_full_unstemmed | Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group |
| title_short | Isolated central nervous system relapses in patients with high-risk neuroblastoma -clinical presentation and prognosis: experience of the Polish Paediatric Solid Tumours Study Group |
| title_sort | isolated central nervous system relapses in patients with high risk neuroblastoma clinical presentation and prognosis experience of the polish paediatric solid tumours study group |
| topic | Central nervous system Clinical course Isolated relapse Metastases Neuroblastoma |
| url | https://doi.org/10.1186/s12885-022-09776-x |
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