Question

Management of inferior vena cava thrombosis in Wilm's tumour.

Hint:

Early diagnosis and prompt surgical intervention are key to managing IVC thrombosis in Wilm’s tumor. A multidisciplinary approach ensures optimal outcomes.

Answer 1

Wilms' tumor (nephroblastoma) is the most common renal malignancy in children. It can be associated with inferior vena cava (IVC) thrombosis, which complicates the management and affects the prognosis. The management of IVC thrombosis in Wilm's tumor requires a multidisciplinary approach involving oncologists, surgeons, and radiologists.
Step 1: Diagnosis of IVC Thrombosis:
- Imaging: The first step in management is accurate diagnosis, typically made using imaging modalities such as ultrasound, CT scan, or MRI. These imaging techniques help to assess the extent of the thrombus and its involvement with surrounding structures, including the renal vein, IVC, and heart.
- Chest X-ray: In cases where the thrombus has extended to the right atrium, a chest X-ray may show evidence of a mass effect on the heart and lungs.
- MRI/CT Venography: These advanced imaging techniques are particularly useful for visualizing the thrombus and planning the surgical approach.
Step 2: Preoperative Management:
- Chemotherapy: Preoperative chemotherapy may be used to shrink the tumor and reduce the size of the thrombus, making it more amenable to surgical resection. A typical chemotherapy regimen for Wilms' tumor includes drugs such as actinomycin D, vincristine, and doxorubicin.
- Anticoagulation: In cases where the thrombus is recent or there is concern for embolization, anticoagulation therapy may be initiated with drugs such as low molecular weight heparin or unfractionated heparin to prevent thrombus propagation.
Step 3: Surgical Management:
- Tumor Resection: The primary treatment for Wilms' tumor is nephrectomy with resection of the tumor. If there is associated IVC thrombosis, the tumor, along with the thrombus, may need to be resected en bloc.
- IVC Thrombectomy: For tumors with extensive thrombus involving the IVC, a thrombectomy procedure may be necessary. This involves removing the thrombus from the IVC while maintaining its integrity. The thrombectomy may be performed in conjunction with the nephrectomy or as a separate procedure, depending on the extent of involvement.
- Cardiac and Vascular Support: In cases where the thrombus has reached the heart or significant vascular involvement is present, cardiothoracic surgeons may be required to assist with the surgical approach and management of the IVC and venous system.
Step 4: Postoperative Care:
- Postoperative Imaging and Monitoring: Following surgery, patients should be monitored with imaging studies to ensure there is no recurrence of thrombus and to assess renal function.
- Chemotherapy Post-Operatively: Adjuvant chemotherapy is typically administered following surgery to address any remaining cancer cells and to prevent recurrence.
- Vascular Follow-Up: Regular follow-up is necessary to ensure that the IVC remains patent and to detect any possible complications, such as deep vein thrombosis or pulmonary embolism.