Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol
Objective: 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 l...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-02-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/13/5/976 |
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| author | Raquel Dávila Fajardo Rhoikos Furtwängler Martine van Grotel Harm van Tinteren Claudia Pasqualini Kathy Pritchard-Jones Reem Al-Saadi Beatriz de Camargo Gema L. Ramírez Villar Norbert Graf Xavier Muracciole Patrick Melchior Daniel Saunders Christian Rübe Marry M. van den Heuvel-Eibrink Geert O. Janssens Arnauld C. Verschuur |
| author_facet | Raquel Dávila Fajardo Rhoikos Furtwängler Martine van Grotel Harm van Tinteren Claudia Pasqualini Kathy Pritchard-Jones Reem Al-Saadi Beatriz de Camargo Gema L. Ramírez Villar Norbert Graf Xavier Muracciole Patrick Melchior Daniel Saunders Christian Rübe Marry M. van den Heuvel-Eibrink Geert O. Janssens Arnauld C. Verschuur |
| author_sort | Raquel Dávila Fajardo |
| collection | DOAJ |
| description | Objective: 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. 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 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. Conclusions: 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. |
| first_indexed | 2024-03-09T00:30:05Z |
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| id | doaj.art-0105d8d4ef194db7a281ed4b6cdf3a6f |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-09T00:30:05Z |
| publishDate | 2021-02-01 |
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| record_format | Article |
| series | Cancers |
| spelling | doaj.art-0105d8d4ef194db7a281ed4b6cdf3a6f2023-12-11T18:31:44ZengMDPI AGCancers2072-66942021-02-0113597610.3390/cancers13050976Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 ProtocolRaquel Dávila Fajardo0Rhoikos Furtwängler1Martine van Grotel2Harm van Tinteren3Claudia Pasqualini4Kathy Pritchard-Jones5Reem Al-Saadi6Beatriz de Camargo7Gema L. Ramírez Villar8Norbert Graf9Xavier Muracciole10Patrick Melchior11Daniel Saunders12Christian Rübe13Marry M. van den Heuvel-Eibrink14Geert O. Janssens15Arnauld C. Verschuur16Department of Radiation Oncology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsDepartment of Paediatric Oncology and Haematology, University Hospital of Saarland, 66421 Homburg, GermanyPrincess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The NetherlandsTrial and Data Center, Princess Maxima Center for Pediatric Oncology, 3584 CS Utrecht, The NetherlandsDepartment of Paediatric Oncology, Institute Gustave Roussy, CEDEX, 94805 Villejuif, FranceDevelopmental Biology & Cancer Research & Teaching Department, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UKDevelopmental Biology & Cancer Research & Teaching Department, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UKResearch Center, Brazilian National Cancer Institute, Rio de Janeiro 20230-240, BrazilDepartment of Paediatric Oncology, Hospital Universitario Virgen del Rocío, 41013 Seville, SpainDepartment of Paediatric Oncology and Haematology, University Hospital of Saarland, 66421 Homburg, GermanyDepartment of Radiation Oncology, Assistance Publique Hôpitaux de Marseille, 13005 Marseille, FranceDepartment of Radiation Oncology, University Hospital of Saarland, 66421 Homburg, GermanyThe Christie NHS Foundation Trust, Manchester M20 4BX, UKDepartment of Radiation Oncology, University Hospital of Saarland, 66421 Homburg, GermanyPrincess Máxima Center for Pediatric Oncology, 3584 CS Utrecht, The NetherlandsDepartment of Radiation Oncology, University Medical Center Utrecht, 3584 CX Utrecht, The NetherlandsDepartment of Paediatric Oncology, La Timone Children’s Hospital, Assistance Publique Hôpitaux de Marseille, 13005 Marseille, FranceObjective: 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. 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 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. Conclusions: 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.https://www.mdpi.com/2072-6694/13/5/976Wilms tumournephroblastomacompletely necroticmetastatic disease |
| spellingShingle | Raquel Dávila Fajardo Rhoikos Furtwängler Martine van Grotel Harm van Tinteren Claudia Pasqualini Kathy Pritchard-Jones Reem Al-Saadi Beatriz de Camargo Gema L. Ramírez Villar Norbert Graf Xavier Muracciole Patrick Melchior Daniel Saunders Christian Rübe Marry M. van den Heuvel-Eibrink Geert O. Janssens Arnauld C. Verschuur Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol Cancers Wilms tumour nephroblastoma completely necrotic metastatic disease |
| title | Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol |
| title_full | Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol |
| title_fullStr | Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol |
| title_full_unstemmed | Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol |
| title_short | Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol |
| title_sort | outcome of stage iv completely necrotic wilms tumour and local stage iii treated according to the siop 2001 protocol |
| topic | Wilms tumour nephroblastoma completely necrotic metastatic disease |
| url | https://www.mdpi.com/2072-6694/13/5/976 |
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