Clinical trial NCT05195411: Vascular Thrombus Involvement in Nephroblastoma
Official title: Nephroblastoma with Vena Cava Thrombosis : Multicenter Study Over 20 Years - Analysis of Key Elements of Surgical Management
Study type: OBSERVATIONAL
Overall status: COMPLETED
Conditions: Nephroblastoma with Vena Cava Thrombosis
Keywords: Nephroblastoma
Wilms tumor
Vena cava thrombosis
Surgical management
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
First posted: 2022-01-19
Enrollment: 72
Sex: ALL
Maximum age (years): 17
Standard ages: CHILD
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: RETROSPECTIVE
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.

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.

Eligibility criteria: 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.

Primary outcomes:
measure: Survival
timeFrame: 22 years
measure: Recurrence
timeFrame: 22 years
measure: Determination of surgical management recommendations
timeFrame: 2 months
Secondary outcomes:
measure: Intraoperative complications
timeFrame: 1 day
measure: Post-operative complications
timeFrame: 30 days
measure: Long-term sequelae
timeFrame: 20 years
measure: Identify the oncological and / or surgical prognostic risk factors
timeFrame: 2 months
Other outcomes:
Locations:
city: Le Kremlin Bicêtre
country: France
facility: Hôpital Kremlin Bicêtre
city: Paris
country: France
facility: Hôpital Necker-Enfants Malades
Study officials:
affiliation: Assistance Publique - Hôpitaux de Paris
name: Sabine Sarnacki, MD, PhD
role: PRINCIPAL_INVESTIGATOR
affiliation: Assistance Publique - Hôpitaux de Paris
name: Luca Pio, MD
role: STUDY_DIRECTOR
References:
citation: 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.