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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

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