Wilms Tumor Rapid Fire: Update Course 2015
Part of
Wilms Tumor 20 items
Educational content from recorded physician discussions — not medical advice. Talk to your (or your child's) care team about your situation.
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What the experts said
The current status for bilateral Wilms tumor with positive lymph nodes and lung metastasis is to start chemotherapy without biopsy.
Tumor shrinkage is defined as a decrease of 50% in tumor size.
The concept with bilateral Wilms tumor is to preserve as much kidney tissue as possible.
If a tumor has not been biopsied and is not responding to chemotherapy, there may be anaplastic elements that would respond to a more aggressive chemotherapy regimen.
Wilms tumors are heterogeneous and biopsy might miss foci of anaplasia.
When bilateral Wilms tumors stop shrinking after chemotherapy, it is usually because of mesenchymal differentiation and not anaplastic tumor.
Bilateral nephron-sparing surgery can be performed surprisingly often in large bilateral Wilms tumors.
The surgical technique for bilateral nephron-sparing involves putting the kidneys on ice, clamping the vessels, then sharp dissection to remove the tumor.
Stage 3 local Wilms tumor requires radiation on both sides.
Recurrence of anaplastic Wilms tumor portends a very bad outcome.
Salvage is difficult for anaplastic Wilms tumor recurrence, despite chemotherapy.
Bilateral Wilms tumors are no longer biopsied upfront; they are treated with chemotherapy first.
Once anaplastic Wilms tumor recurs, it is very hard to salvage.
Multifocal Wilms tumors raise concern about the underlying embryology of the kidney and risk for developing additional tumors.
Mutations in WT1 or WT2 increase risk for additional Wilms tumors.
97% of large bilateral kidney tumors in children are Wilms tumor.
Upfront chemotherapy without biopsy is appropriate for bilateral kidney tumors because only a very small percentage are not Wilms tumor.
Bob Shamberger has made the point that if a Wilms tumor is not responding to chemotherapy, tissue is needed to check for anaplasia.
Most pathologists think anaplasia in Wilms tumor is present primarily rather than induced by chemotherapy.