Vascular Thrombus Involvement in Nephroblastoma

Study Overview
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Brief Summary
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Nephroblastoma (Wilms tumor) is the most common kidney tumor in children. It is a malignant embryonic tumor with a good prognosis with more than 85% long-term survival with appropriate chemotherapy, surgery (which most often consists of a total nephrectomy) and radiotherapy for locally invasive forms. Some nephroblastomas (approximately 10%) present with vascular extension with vena cava thrombus, a situation which may worsen the prognosis due to the complexity of the surgery. While the oncological treatment of nephroblastoma is highly formalized, to date there is no specific guideline on the surgical management of this rare clinical presentation of nephroblastomas. The aim of the study is to provide recommendations for the surgical management of nephroblastomas with vena cava thrombus in a large multicenter series.

Detailed Description
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Nephroblastoma (Wilms tumor) is the most common kidney tumor in children. It is a malignant embryonic tumor with a good prognosis with more than 85% long-term survival with appropriate chemotherapy, surgery (which most often consists of a total nephrectomy) and radiotherapy for locally invasive forms. Some nephroblastomas (approximately 10%) present with vascular extension with vena cava thrombus, a situation which may worsen the prognosis due to the complexity of the surgery. While the oncological treatment of nephroblastoma is highly formalized, to date there is no specific guideline on the surgical management of this rare clinical presentation of nephroblastomas. The management of children with nephroblastomas with vena cava thrombosis is very specific because of the vital risks to diagnosis, especially pulmonary embolism but also cardiac arrest (in the event of extension in the right atrium) and Cerebrovascular accidents by embolism, rarer. Surgery of the primary tumor and its intravascular extension is complex and may require the use of extracorporeal circulation, a source of significant morbidity and even mortality. These patients who will actually have a single kidney are at high risk for renal failure due to possible contralateral kidney damage during surgery. Surgery is not clearly codified because cases are rare, including in expert centers. The aim of the study is to provide recommendations for the surgical management of nephroblastomas with vena cava thrombus in a large multicenter series.

Official Title
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Nephroblastoma With Vena Cava Thrombosis : Multicenter Study Over 20 Years - Analysis of Key Elements of Surgical Management

Conditions
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Nephroblastoma With Vena Cava Thrombosis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Minors with nephroblastoma with vena cava thrombosis +/- atrial extension. Cared between 1999 and 2019 in the centers of the study. Exclusion Criteria: - Other renal and non-renal tumors with vena cava thrombosis.

Ages Eligible for Study
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Maximum Age: 17 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Nephroblastoma with vena cava thrombosis<br>Children with nephroblastoma with vena cava thrombosis +/- atrial extension, cared between 1999 and 2019 in Parisian hospitals: Necker and Bicêtre. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Survival | Study of the medical file. | 22 years | 
| Recurrence | Study of the medical file. Local or metastatic recurrence. | 22 years | 
| Determination of surgical management recommendations | Study of the medical file. Determination of surgical management recommendations based on survival, the occurrence of local or metastatic recurrence and the study of medical care of the patient. | 2 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Intraoperative complications | Study of the medical file. Description of intraoperative complications and Dindo-Clavien classification (classification that defines and grades operative complications in 10 grades). | 1 day | 
| Post-operative complications | Study of the medical file. Description of post-operative complications and Dindo-Clavien classification (classification that defines and grades operative complications in 10 grades). | 30 days | 
| Long-term sequelae | Study of the medical file. Sequelae linked to the toxicity of chemotherapy, radiotherapy and surgery. | 20 years | 
| Identify the oncological and / or surgical prognostic risk factors | Study of the medical file. Identify the oncological and / or surgical prognostic risk factors depending on the medical treatment received. | 2 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Nephroblastoma, Wilms tumor, Vena cava thrombosis, Surgical management