Nephroblastoma With Vena Cava Thrombosis
Conditions
Keywords
Nephroblastoma, Wilms tumor, Vena cava thrombosis, Surgical management
Brief summary
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
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.
Interventions
None listed
Sponsors
URC-CIC Paris Descartes Necker Cochin
Assistance Publique - Hôpitaux de Paris
Study design
Observational model
COHORT
Time perspective
RETROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
No minimum to 17 Years
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Survival | 22 years | Study of the medical file. |
| Recurrence | 22 years | Study of the medical file. Local or metastatic recurrence. |
| Determination of surgical management recommendations | 2 months | 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. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Intraoperative complications | 1 day | Study of the medical file. Description of intraoperative complications and Dindo-Clavien classification (classification that defines and grades operative complications in 10 grades). |
| Identify the oncological and / or surgical prognostic risk factors | 2 months | Study of the medical file. Identify the oncological and / or surgical prognostic risk factors depending on the medical treatment received. |
| Post-operative complications | 30 days | Study of the medical file. Description of post-operative complications and Dindo-Clavien classification (classification that defines and grades operative complications in 10 grades). |
| Long-term sequelae | 20 years | Study of the medical file. Sequelae linked to the toxicity of chemotherapy, radiotherapy and surgery. |
Countries
France
Outcome results
None listed