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Characteristics and survival of patients with Wilms tumor and bone metastases: a SIOP-RTSG 93-01, SIOP 2001, and UK-IMPORT Study

Background Wilms tumor (WT) has an excellent outcome. Studies suggest that patients with WT and bone metastases (WT-BM) have a poor prognosis. This analysis describes the characteristics and outcome of patients with WT-BM, registered by the International Society of Paediatric Oncology—Renal Tumor St...

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Autori principali: Tagarelli, Arianna (Autore) , Dandis, Rana (Autore) , Van Tinteren, Harm (Autore) , Furtwängler, Rhoikos (Autore) , Al-Saadi, Reem (Autore) , Chowdhury, Tanzina (Autore) , Ramirez-Villar, Gema Lucia (Autore) , de Camargo, Beatriz (Autore) , Schenk, Jens-Peter (Autore) , Teixeira, Roberto Augusto Plaza (Autore) , van Grotel, Martine (Autore) , Brok, Jesper (Autore) , Brisse, Herve J. (Autore) , Serra, Annalisa (Autore) , Graf, Norbert (Autore) , Verschuur, Arnauld (Autore) , van den Heuvel-Eibrink, Marry M. (Autore)
Natura: Article (Journal)
Lingua:inglese
Pubblicazione: July 2026
In: Pediatric blood & cancer
Year: 2026, Volume: 73, Fascicolo: 7, Pages: 1-7
ISSN:1545-5017
DOI:10.1002/1545-5017.70270
Accesso online:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1002/1545-5017.70270
Verlag, lizenzpflichtig, Volltext: https://onlinelibrary.wiley.com/doi/abs/10.1002/1545-5017.70270
Testo
Note sull'autore:Arianna Tagarelli, Rana Dandis, Harm Van Tinteren, Rhoikos Furtwängler, Reem Al-Saadi, Tanzina Chowdhury, Gema Lucia Ramirez-Villar, Beatriz de Camargo, Jens-Peter Schenk, Roberto Augusto Plaza Teixeira, Martine van Grotel, Jesper Brok, Herve J. Brisse, Annalisa Serra, Norbert Graf, Arnauld Verschuur, Marry M. van den Heuvel-Eibrink
Descrizione
Riassunto:Background Wilms tumor (WT) has an excellent outcome. Studies suggest that patients with WT and bone metastases (WT-BM) have a poor prognosis. This analysis describes the characteristics and outcome of patients with WT-BM, registered by the International Society of Paediatric Oncology—Renal Tumor Study Group (SIOP-RTSG) and UK-IMPORT STUDY. Methods We retrospectively assessed clinical characteristics and outcome of patients with WT-BM at diagnosis treated according to SIOP-RTSG 93-01, SIOP 2001/UK-IMPORT study protocols. Results Among 7399 patients diagnosed with WT between 1993 and 2019, 47 were identified with WT-BM (12 males/35 females), accounting for 3.3% of stage IV-WT. The median age at diagnosis was 63 months (24-292 months). Four patients (9%) had only BM involvement and 43 patients (91%) had multisite metastases. Local stage was I-II in 15 (32%) patients, stage III in 26 (55%) patients, and unknown in 6 (13%) patients. Preoperative chemotherapy consisted of three drugs in 95% of cases with available data (38/40). Histological subtyping identified low-risk (LR, n = 4), intermediate-risk (IR, n = 29), high-risk (HR, n = 7) tumors, unknown/others in seven patients. The majority had postoperative treatment according to protocol. Among 35 patients with data, 33 (94%) received any kind of radiotherapy. EFS and OS were 67% and 74% at 2 years, decreasing to 64% and 62% at five years. EFS at 2 years was 14% in the HR group and 84% in the IR group. Conclusions WT-BM is a rare condition. Awareness of its existence and multimodality treatment are warranted.
Descrizione del documento:Zuerst veröffentlicht: 20. April 2026
Gesehen am 16.06.2026
Descrizione fisica:Online Resource
ISSN:1545-5017
DOI:10.1002/1545-5017.70270

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