%0 Journal Article
%A Groenendijk, Alissa
%A van Tinteren, Harm
%A Jiang, Yilin
%A de Krijger, Ronald R
%A Vujanic, Gordan M
%A Godzinski, Jan
%A Rübe, Christian
%A Schenk, Jens-Peter
%A Morosi, Carlo
%A Pritchard-Jones, Kathy
%A Al-Saadi, Reem
%A Vaidya, Sucheta J
%A Verschuur, Arnauld C
%A Ramírez-Villar, Gema L
%A Graf, Norbert
%A de Camargo, Beatriz
%A Drost, Jarno
%A Perotti, Daniela
%A van den Heuvel-Eibrink, Marry M
%A Brok, Jesper
%A Spreafico, Filippo
%A Mavinkurve-Groothuis, Annelies M C
%T Outcome of SIOP patients with low- or intermediate-risk Wilms tumour relapsing after initial vincristine and actinomycin-D therapy only - the SIOP 93-01 and 2001 protocols.
%D 2022
%U http://hdl.handle.net/10668/22171
%X Society of International Pediatric Oncology - Renal Tumor Study Group (SIOP-RTSG) treatment recommendations for relapsed Wilms tumour (WT) are stratified by the intensity of first-line treatment. To explore the evidence for the treatment of patients relapsing after vincristine and actinomycin-D (VA) treatment for primary WT, we retrospectively evaluated rescue treatment and survival of this patient group. We included 109 patients with relapse after VA therapy (no radiotherapy) for stage I-II primary low- or intermediate-risk WT from the SIOP 93-01 and SIOP 2001 studies. Univariate Cox regression analysis was performed to study the effect of relapse treatment intensity on event-free survival (EFS) and overall survival (OS). Relapse treatment intensity was classified into vincristine, actinomycin-D, and either doxorubicin or epirubicin (VAD), and more intensive therapies (ifosfamide/carboplatin/etoposide [ICE]/≥ 4 drugs/high-dose chemotherapy with haematopoietic stem cell transplantation [HD HSCT]). Relapse treatment regimens included either VAD, or cyclophosphamide/carboplatin/etoposide/doxorubicin (CyCED), or ICE backbones. Radiotherapy was administered in 62 patients and HD HSCT in 15 patients. Overall, 5-year EFS and OS after relapse were 72.3% (95% confidence interval [CI]: 64.0-81.6%) and 79.3% (95% CI: 71.5-88.0%), respectively. Patients treated with VAD did not fare worse when compared with patients treated with more intensive therapies (hazard ratio EFS: 0.611 [95% CI: 0.228-1.638] [p-value = 0.327] and hazard ratio OS: 0.438 [95% CI: 0.126-1.700] [p-value = 0.193]). Patients with relapsed WT after initial VA-only treatment showed no inferior EFS and OS when treated with VAD regimens compared with more intensive rescue regimens. A subset of patients relapsing after VA may benefit from less intensive rescue treatment than ICE/CyCED-based regimens and deserve to be pinpointed by identifying additional (molecular) prognostic factors in future studies.
%K Recurrence
%K SIOP protocol
%K Treatment outcome
%K Wilms tumour
%~