RT Journal Article
T1 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.
A1 van der Beek, Justine N
A1 Hol, Janna A
A1 Coulomb-l'Hermine, Aurore
A1 Graf, Norbert
A1 van Tinteren, Harm
A1 Pritchard-Jones, Kathy
A1 Houwing, Maite E
A1 de Krijger, Ronald R
A1 Vujanic, Gordan M
A1 Dzhuma, Kristina
A1 Schenk, Jens-Peter
A1 Littooij, Annemieke S
A1 Ramírez-Villar, Gema L
A1 Murphy, Dermot
A1 Ray, Satyajit
A1 Al-Saadi, Reem
A1 Gessler, Manfred
A1 Godzinski, Jan
A1 Ruebe, Christian
A1 Collini, Paola
A1 Verschuur, Arnaud C
A1 Frisk, Tony
A1 Vokuhl, Christian
A1 Hulsbergen-van de Kaa, Christina A
A1 de Camargo, Beatriz
A1 Sandstedt, Bengt
A1 Selle, Barbara
A1 Tytgat, Godelieve A M
A1 van den Heuvel-Eibrink, Marry M
K1 pediatric
K1 renal cell carcinoma
K1 survival
K1 treatment
AB 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.
YR 2021
FD 2021-02-03
LK http://hdl.handle.net/10668/16987
UL http://hdl.handle.net/10668/16987
LA en
DS RISalud
RD Apr 7, 2025