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doi:10.22028/D291-40615
Titel: | Clear cell sarcoma of the kidney in Austrian children: Long-term survival after relapse |
VerfasserIn: | Friesenbichler, Waltraud Lüftinger, Roswitha Kropshofer, Gabriele Henkel, Martin Amann, Gabriele Furtwängler, Rhoikos Graf, Norbert Kager, Leo |
Sprache: | Englisch |
Titel: | Pediatric Blood & Cancer |
Bandnummer: | 68 |
Heft: | 5 |
Verlag/Plattform: | Wiley |
Erscheinungsjahr: | 2021 |
Freie Schlagwörter: | central nervous system metastases clear cell sarcoma of the kidney relapse treatment renal tumour |
DDC-Sachgruppe: | 610 Medizin, Gesundheit |
Dokumenttyp: | Journalartikel / Zeitschriftenartikel |
Abstract: | Introduction: Clear cell sarcoma of the kidney (CCSK) is a rare malignant childhood renal tumour. Recently, the central nervous system (CNS) was found to be the most frequent site of relapse associated with a poor outcome. Optimal treatment strategies are scarce. Patients and Methods: Retrospective data analysis of all Austrian children with CCSK. They were enrolled in the Austrian-Hungarian Wilms Tumour Study (AHWTS) 1989, the SIOP93-01 or the SIOP2001 study between 1990 and 2019. Demographic, diagnostic, treatment-related variables and survival data were analysed. Results: We identified 12 children with CCSK (M = 7, F = 5; median age 1.6 years). All had localised disease (stage I: 2; stage II: 2; stage III: 8) at diagnosis, and a first complete remission (CR1) was achieved in 12/12. Six patients are in an ongoing CR1 (median follow-up 10 years). Six other patients had a relapse (local 1; brain 5) a median time of 2.4 years from diagnosis. Two patients died of the disease 4 months and 2.8 years after first relapse. Four of five patients with CNS relapse are in CR2 with a median followup time of 9.3 years after relapse diagnosis. Relapse treatment included a combination of chemotherapy, radiation and surgery. Two children received high-dose chemotherapy followed by autologous stem cell rescue, and one child received intrathecal mafosphamide. Long-term side effects after treatment were impaired tubular renal function (n = 4), cardiomyopathy (n = 1) and growth disorders (n = 1). Conclusions: In this series, the brain was the most common site of relapse. Long-term survival after recurrence was achievable with intensive multimodal therapy. |
DOI der Erstveröffentlichung: | 10.1002/pbc.28860 |
URL der Erstveröffentlichung: | https://doi.org/10.1002/pbc.28860 |
Link zu diesem Datensatz: | urn:nbn:de:bsz:291--ds-406152 hdl:20.500.11880/36493 http://dx.doi.org/10.22028/D291-40615 |
ISSN: | 1545-5017 1545-5009 |
Datum des Eintrags: | 27-Sep-2023 |
Fakultät: | M - Medizinische Fakultät |
Fachrichtung: | M - Pädiatrie |
Professur: | M - Prof. Dr. Norbert Graf |
Sammlung: | SciDok - Der Wissenschaftsserver der Universität des Saarlandes |
Dateien zu diesem Datensatz:
Datei | Beschreibung | Größe | Format | |
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Pediatric Blood Cancer - 2021 - Friesenbichler.pdf | 207,26 kB | Adobe PDF | Öffnen/Anzeigen |
Diese Ressource wurde unter folgender Copyright-Bestimmung veröffentlicht: Lizenz von Creative Commons