RT Journal Article
T1 Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol.
A1 Dávila Fajardo, Raquel
A1 Furtwängler, Rhoikos
A1 van Grotel, Martine
A1 van Tinteren, Harm
A1 Pasqualini, Claudia
A1 Pritchard-Jones, Kathy
A1 Al-Saadi, Reem
A1 de Camargo, Beatriz
A1 Ramírez Villar, Gema L
A1 Graf, Norbert
A1 Muracciole, Xavier
A1 Melchior, Patrick
A1 Saunders, Daniel
A1 Rübe, Christian
A1 van den Heuvel-Eibrink, Marry M
A1 Janssens, Geert O
A1 Verschuur, Arnauld C
K1 Wilms tumour
K1 completely necrotic
K1 metastatic disease
K1 nephroblastoma
AB Wilms tumour (WT) patients with a localised completely necrotic nephroblastoma after preoperative chemotherapy are a favourable outcome group. Since the introduction of the SIOP 2001 protocol, the SIOP- Renal Tumour Study Group (SIOP-RTSG) has omitted radiotherapy for such patients with low-risk, local stage III in an attempt to reduce treatment burden. However, for metastatic patients with local stage III, completely necrotic WT, the recommendations led to ambiguous use. The purpose of this descriptive study is to demonstrate the outcomes of patients with metastatic, completely necrotic and local stage III WT in relation to the application of radiotherapy or not. all metastatic patients with local stage III, completely necrotic WT after 6 weeks of preoperative chemotherapy who were registered in the SIOP 2001 study were included in this analysis. The pattern of recurrence according to the usage of radiation treatment and 5 year event-free survival (EFS) and overall survival (OS) was analysed. seven hundred and three metastatic WT patients were registered in the SIOP 2001 database. Of them, 47 patients had a completely necrotic, local stage III WT: 45 lung metastases (11 combined localisations), 1 liver/peritoneal, and 1 tumour thrombus in the renal vein and the inferior vena cava with bilateral pulmonary arterial embolism. Abdominal radiotherapy was administered in 29 patients (62%; 29 flank/abdominal irradiation and 9 combined with lung irradiation). Eighteen patients did not receive radiotherapy. Median follow-up was 6.6 years (range 1-151 months). Two of the 47 patients (4%) developed disease recurrence in the lung (one combined with abdominal relapse) and eventually died of the disease. Both patients had received abdominal radiotherapy, one of them combined with lung irradiation. Five-year EFS and OS were 95% and 95%, respectively. the outcome of patients with stage IV, local stage III, completely necrotic Wilms tumours is excellent. Our results suggest that abdominal irradiation in this patient category may not be of added value in first-line treatment, consistent with the current recommendation in the SIOP-RTSG 2016 UMBRELLA protocol.
SN 2072-6694
YR 2021
FD 2021-02-26
LK http://hdl.handle.net/10668/17260
UL http://hdl.handle.net/10668/17260
LA en
DS RISalud
RD Apr 19, 2025