TY - JOUR
T1 - Outcome of stage iv completely necrotic wilms tumour and local stage iii treated according to the siop 2001 protocol
AU - Fajardo, Raquel Dávila
AU - Furtwängler, Rhoikos
AU - van Grotel, Martine
AU - van Tinteren, Harm
AU - Pasqualini, Claudia
AU - Pritchard-Jones, Kathy
AU - Al-Saadi, Reem
AU - de Camargo, Beatriz
AU - Villar, Gema L.Ramírez
AU - Graf, Norbert
AU - Muracciole, Xavier
AU - Melchior, Patrick
AU - Saunders, Daniel
AU - Rübe, Christian
AU - van den Heuvel-Eibrink, Marry M.
AU - Janssens, Geert O.
AU - Verschuur, Arnauld C.
N1 - Funding Information:
Acknowledgments: The SIOP WT 2001 study was funded by Cancer Research UK (grant C1188/A8687), the UK National Cancer Research Network and Children’s Cancer and Leukaemia Group (CCLG) (who supported the UK section), Société Française des Cancers de l’Enfant and Association Leon Berard Enfant Cancéreux and Enfant et Santé (who supported the French section), Gesellschaft für Pädiatrische Onkologie und Hämatologie and Deutsche Krebschilfe (grant 50-2709-Gr2, who supported the German section), Grupo Cooperativo Brasileiro para o Tratamento do Tumor de Wilms and Sociedade Brasileira de Oncologia Pediátrica (who supported the Brazilian section), and the Spanish Society of Pediatric Haematology and Oncology and the Spanish Association Against Cancer (who supported the Spanish section), and SIOP-NL. K P-J is partly supported by the National Institute for Health Research Biomedical Research Centre Funding Scheme. We acknowledge the enormous efforts made by more than 1000 clinicians, radiologists, and pathologists working at the 251 childhood cancer treatment centres in this study from 28 countries who enrolled and followed up with patients in this study, and the patients and their families for their participation.
Publisher Copyright:
© 2021 by the authors. Licensee MDPI, Basel, Switzerland.
PY - 2021/3
Y1 - 2021/3
N2 - Objective: Wilms tumour (WT) patients with a localised completely necrotic nephroblas-toma 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. How-ever, 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. Methods and materials: 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. Results: 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 localisa-tions), 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. Conclu-sions: 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.
AB - Objective: Wilms tumour (WT) patients with a localised completely necrotic nephroblas-toma 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. How-ever, 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. Methods and materials: 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. Results: 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 localisa-tions), 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. Conclu-sions: 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.
KW - Completely necrotic
KW - Metastatic disease
KW - Nephroblastoma
KW - Wilms tumour
KW - nephroblastoma
KW - metastatic disease
KW - completely necrotic
UR - http://www.scopus.com/inward/record.url?scp=85101570907&partnerID=8YFLogxK
U2 - 10.3390/cancers13050976
DO - 10.3390/cancers13050976
M3 - Article
C2 - 33652659
AN - SCOPUS:85101570907
SN - 2072-6694
VL - 13
SP - 1
EP - 10
JO - Cancers
JF - Cancers
IS - 5
M1 - 976
ER -