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Outcome of Stage IV Completely Necrotic Wilms Tumour and Local Stage III Treated According to the SIOP 2001 Protocol

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  • معلومة اضافية
    • Contributors:
      Princess Máxima Center for Pediatric Oncology; University Medical Center [Utrecht]; Saarland University Hospital (UKS); Département de cancérologie de l'enfant et de l'adolescent [Gustave Roussy]; Institut Gustave Roussy (IGR); Great Ormond Street Institute of Child Health (UCL); University College of London [London] (UCL); Instituto Nacional de Câncer [Rio de Janeiro] (INCA); Hospital Universitario Virgen del Rocío [Sevilla]; Méthodes computationnelles pour la prise en charge thérapeutique en oncologie : Optimisation des stratégies par modélisation mécaniste et statistique (COMPO); Inria Sophia Antipolis - Méditerranée (CRISAM); Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre de Recherche en Cancérologie de Marseille (CRCM); Aix Marseille Université (AMU)-Institut Paoli-Calmettes; Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU)-Institut Paoli-Calmettes; Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS); Service de radiothérapie - [Hôpital de la Timone - Hôpital Nord - APHM]; Hôpital de la Timone [CHU - APHM] (TIMONE)- Hôpital Nord [CHU - APHM]; Centre de Recherche en Cancérologie de Marseille (CRCM); The Christie NHS Foundation Trust [Manchester, Royaume-Uni]; Pédiatrie et oncologie pédiatrique [Hôpital de la Timone - APHM]; Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)- Hôpital de la Timone [CHU - APHM] (TIMONE); Department of Radiotherapy [Utrecht]; Princess Máxima Center for Pediatric Oncology [Utrecht, Pays-Bas]; Department of Paediatric Oncology and Haematology; Saarland University Hospital; Trial and Data Center; Princess Máxima Center for Pediatric Oncology [Utrecht, Netherlands]; Developmental Biology & Cancer Research & Teaching Department; UCL Great Ormond Street Institute of Child Health; Partenaires INRAE-Partenaires INRAE; Unidad de Oncología Pediátrica
    • بيانات النشر:
      MDPI AG, 2021.
    • الموضوع:
      2021
    • نبذة مختصرة :
      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.
    • File Description:
      application/pdf
    • ISSN:
      2072-6694
    • Rights:
      OPEN
    • الرقم المعرف:
      edsair.doi.dedup.....13f03f172cd534d56572671ff42d89f3