Vascular Thrombus Involvement in Nephroblastoma (VIN)

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

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.

Study Overview

• Status: Completed
• Conditions: Nephroblastoma With Vena Cava Thrombosis

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.

• Study Type: Observational
• Enrollment (Actual): 72

Contacts and Locations

Study Locations

• France
  • Le Kremlin-Bicêtre, France, 94270
    • Hôpital Kremlin Bicêtre
  • Paris, France, 75015
    • Hôpital Necker-Enfants Malades

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: No older than 17 years (Child)
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

Children with nephroblastoma with vena cava thrombosis +/- atrial extension cared between 1999 and 2019 in Parisian hospitals: Necker and Bicêtre.

Description

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.

Study Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort
Nephroblastoma with vena cava thrombosis; 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?

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

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Collaborators: URC Necker Cochin, France
• Investigators: Principal Investigator: Sabine Sarnacki, MD, PhD, Assistance Publique - Hopitaux de Paris; Study Director: Luca Pio, MD, Assistance Publique - Hopitaux de Paris

Publications and helpful links

General Publications

• Pio L, Abib S, Guerin F, Chardot C, Blanc T, Sarrai N, Martelli H, De Souza FKM, Fanelli MCA, Tamisier D, Guilhen JCS, Le Bret E, Belli E, Fadel E, Cypriano MDS, Minard V, Pasqualini C, Schleiermacher G, Lemelle L, Rod J, Irtan S, Pistorio A, Gauthier F, Branchereau S, Sarnacki S. Surgical Management of Wilms Tumors with Intravenous Extension: A Multicenter Analysis of Clinical Management with Technical Insights. Ann Surg Oncol. 2024 Jul;31(7):4713-4723. doi: 10.1245/s10434-024-15232-w. Epub 2024 Apr 5.

Study record dates

Study Major Dates

• Study Start (Actual): December 12, 2022
• Primary Completion (Actual): March 15, 2023
• Study Completion (Actual): March 15, 2023

Study Registration Dates

• First Submitted: January 4, 2022
• First Submitted That Met QC Criteria: January 4, 2022
• First Posted (Actual): January 19, 2022

Study Record Updates

• Last Update Posted (Estimated): September 12, 2025
• Last Update Submitted That Met QC Criteria: September 5, 2025
• Last Verified: November 1, 2023

More Information

Terms related to this study

• Keywords: Wilms tumor; Surgical management; Nephroblastoma; Vena cava thrombosis
• Additional Relevant MeSH Terms: Urogenital Diseases; Urogenital Neoplasms; Neoplasms by Site; Neoplasms; Male Urogenital Diseases; Kidney Diseases; Urologic Diseases; Female Urogenital Diseases; Female Urogenital Diseases and Pregnancy Complications; Genetic Diseases, Inborn; Neoplasms by Histologic Type; Urologic Neoplasms; Kidney Neoplasms; Neoplastic Syndromes, Hereditary; Neoplasms, Complex and Mixed; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Wilms Tumor
• Other Study ID Numbers: APHP201331

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No