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Titel: Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93-01, 2001 and UK-IMPORT database: A report of the SIOP-Renal Tumor Study Group
VerfasserIn: van der Beek, Justine N.
Hol, Janna A.
Coulomb-l'Hermine, Aurore
Graf, Norbert
van Tinteren, Harm
Pritchard-Jones, Kathy
Houwing, Maite E.
de Krijger, Ronald R.
Vujanic, Gordan M.
Dzhuma, Kristina
Schenk, Jens-Peter
Littooij, Annemieke S.
Ramírez-Villar, Gema L.
Murphy, Dermot
Ray, Satyajit
Al-Saadi, Reem
Gessler, Manfred
Godzinski, Jan
Ruebe, Christian
Collini, Paola
Verschuur, Arnaud C.
Frisk, Tony
Vokuhl, Christian
Hulsbergen-van de Kaa, Christina A.
de Camargo, Beatriz
Sandstedt, Bengt
Selle, Barbara
Tytgat, Godelieve A. M.
van den Heuvel-Eibrink, Marry M.
Sprache: Englisch
Titel: International Journal of Cancer
Bandnummer: 148
Heft: 11
Seiten: 2724-2735
Verlag/Plattform: Wiley
Erscheinungsjahr: 2021
Freie Schlagwörter: pediatric
renal cell carcinoma
survival
treatment
DDC-Sachgruppe: 610 Medizin, Gesundheit
Dokumenttyp: Journalartikel / Zeitschriftenartikel
Abstract: In children, renal cell carcinoma (RCC) is rare. This study is the first report of pediatric patients with RCC registered by the International Society of Pediatric Oncology-Renal Tumor Study Group (SIOP-RTSG). Pediatric patients with histologically confirmed RCC, registered in SIOP 93-01, 2001 and UK-IMPORT databases, were included. Event-free survival (EFS) and overall survival (OS) were analyzed using the Kaplan-Meier method. Between 1993 and 2019, 122 pediatric patients with RCC were registered. Available detailed data (n = 111) revealed 56 localized, 30 regionally advanced, 25 metastatic and no bilateral cases. Histological classification according to World Health Organization 2004, including immunohistochemical and molecular testing for transcription factor E3 (TFE3) and/or EB (TFEB) translocation, was available for 65/122 patients. In this group, the most common histological subtypes were translocation type RCC (MiT-RCC) (36/64, 56.3%), papillary type (19/64, 29.7%) and clear cell type (4/64, 6.3%). One histological subtype was not reported. In the remaining 57 patients, translocation testing could not be performed, or TFE-cytogenetics and/or immunohistochemistry results were missing. In this group, the most common RCC histological subtypes were papillary type (21/47, 44.7%) and clear cell type (11/47, 23.4%). Ten histological subtypes were not reported. Estimated 5-year (5y) EFS and 5y OS of the total group was 70.5% (95% CI = 61.7%- 80.6%) and 84.5% (95% CI = 77.5%-92.2%), respectively. Estimated 5y OS for localized, regionally advanced, and metastatic disease was 96.8%, 92.3%, and 45.6%, respectively. In conclusion, the registered pediatric patients with RCC showed a reasonable outcome. Survival was substantially lower for patients with metastatic disease. This descriptive study stresses the importance of full, prospective registration including TFE-testing.
DOI der Erstveröffentlichung: 10.1002/ijc.33476
URL der Erstveröffentlichung: https://doi.org/10.1002/ijc.33476
Link zu diesem Datensatz: urn:nbn:de:bsz:291--ds-406141
hdl:20.500.11880/36492
http://dx.doi.org/10.22028/D291-40614
ISSN: 1097-0215
0020-7136
Datum des Eintrags: 27-Sep-2023
Bezeichnung des in Beziehung stehenden Objekts: Supporting Information
In Beziehung stehendes Objekt: https://onlinelibrary.wiley.com/action/downloadSupplement?doi=10.1002%2Fijc.33476&file=ijc33476-sup-0001-supinfo.pdf
Fakultät: M - Medizinische Fakultät
Fachrichtung: M - Pädiatrie
M - Radiologie
Professur: M - Prof. Dr. Norbert Graf
M - Prof. Dr. Christian Rübe
Sammlung:SciDok - Der Wissenschaftsserver der Universität des Saarlandes

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