Outcome of stage IV totally necrotic Wilms tumour and local stage III treated according to the SIOP-2001 protocol
Fajardo, R. D. Furtwangler, R. Van Tinteren, H. Pasqualini, C. Pritchard-Jones, K. Bergeron, C. De Camargo, B. Villar, G. L. R. Van den Heuvel-Eibrink, M. M. Graf, N.
Fajardo, R. D.
Furtwangler, R.
Van Tinteren, H.
Pasqualini, C.
Pritchard-Jones, K.
Bergeron, C.
De Camargo, B.
Villar, G. L. R.
Van den Heuvel-Eibrink, M. M.
Graf, N.
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Abstract
Background and Aims: Paediatric patients with a metastatic renal
tumour and totally necrotic nephroblastoma or Wilms tumour (TN-
WT) after preoperative chemotherapy have a very good outcome.
In order to reduce treatment burden, SIOP-RTSG has abolished
radiotherapy for metastatic patients with low-risk stage III since
the introduction of the SIOP-2001 protocol. The purpose of this
descriptive study is to demonstrate outcome of patients with stage
IV, TN, local stage III WT, treated according to the SIOP-2001
protocol.
Methods: All metastatic patients with local stage III, TN-WT after 6
weeks of preoperative chemotherapy, registered in the SIOP-2001
study were included in this analysis. The pattern of recurrence accord-
ing to applied radiation treatment and 5-year loco-regional control
(LRC), event-free survival (EFS) and overall survival (OS) were anal-
ysed.
Results: Of the 703 metastatic WT patients registered in the SIOP-
2001 protocol, 48 patients had a TN, local stage III WT (46 lung
metastasis (12 combined with other localization), 1 liver/abdomen
and 1 other). Adjuvant abdominal radiotherapy was administered in
27 patients (56%) (27 flank/abdominal irradiation, 8 combined with
lung irradiation). Twenty-one patients were not irradiated. Median
follow-up was 82 months (range 1-151 months). Two of the 48
patients (4%) developed disease recurrence (both lung, one com-
bined with abdominal relapse) and eventually died. Both patients
had received abdominal radiotherapy, 1 of them combined with
lung irradiation. None of the patients treated without radiother-
apy relapsed. Five-year LRC, EFS and OS were 98%, 95% and 95%,
respectively.
Conclusions: Outcome of patients with stage IV and locally stage III,
totally necrotic Wilms tumours seems to be excellent. Our results
suggest that irradiation in this patient category does not have any
added value in first-line treatment, as recommended in the SIOP RTSG-
UMBRELLA protocol.
Description
Date
2020
Publisher
Collections
Keywords
Type
Meetings and Proceedings
Citation
Fajardo RD, Furtwangler R, Van Tinteren H, Pasqualini C, Pritchard-Jones K, Bergeron C, et al. Outcome of stage IV totally necrotic Wilms tumour and local stage III treated according to the SIOP-2001 protocol. Pediatric Blood & Cancer. 2020;67:S66-S