Update Course Rewind: Updates in Wilms Management 2024

Surgery first or chemo first? Would you risk changing the order for Wilm's tumor patients if it could change the outcome? We discuss it here in today's video. Hello pediatric surgery family. I'm Lizzie Lee from Cincinnati Children's Hospital Medical Center. Today, we will dive into new approaches for Wilm's tumor management with Dr. Badrara Mullapudi from Children's Mercy Kansas City. Let's see our first case. We have a 19-month old male presents with a newly noted right-sided abdominal swelling, decreased PO intake. Ultrasound, CT scan, very suspicious for Wilm's. The patient's imaging is classic for Wilm's. Let's see the poll results from the combined live and virtual audience. Most would do A. Most of the audience would do a resection with a close second choice being chemotherapy. When you look at the audience poll, the reason it was pretty split is because this is an international poll. Our European colleagues would offer chemotherapy, no biopsy, and of course that's where the data differ. If it was strictly in North America or COG sites, then it would be very much a resection at diagnosis. This highlights the key differences between treatment approaches worldwide, which include the COG or Children's Oncology Group and the Siop, Society of Pediatric Oncology guidelines. It also would impact the chemotherapy answer because you would add doxorubicin even for a low, stage tumor. Exactly. Uh, so looking at the clinical scenario, we did successfully resect that. Margins were negative. Mass weighs about 728 grams. Pathology showed Blastemal local stage two Wilm's tumor because it involves the renal sinus lymphovascular spaces. Overall, this histology is favorable. Cytogenetics, it's negative for loss of heterozygosity, 1p16q. What do we need to know about loss of heterozygosity and what significance of gain of one? Heterozygosity is the loss of genetic diversity in the tumor cells. In Wilm's tumor, this can indicate more aggressive disease. Gain of 1q refers to an extra copy of a section of chromosome 1, which is linked to worse outcomes in some cancers. Let's see what the audience thinks would be the next best step. The majority of the audience chose to give vincristine and dactinomycin. I would go with C2. I think the one reason you would add doxorubicin to keep in mind, have loss of heterozygosity at 1p and 16q. That is important to remember because that's the one that shows higher recurrence when you have loss of heterozygosity. Let's switch up the parameters of the clinical scenario. Now the tumor is stage one, weighs 450 grams and has loss of heterozygosity. How will we approach this differently? For the surgeons, there's going to be a lot more of waiting for the biology before you determine if chemotherapy is needed. As you're thinking about placing the port, there's going to be more of wait and see what all these biologic markers look like. Absolutely, and that's how your patient family discussion should continue. Not just saying, if you're less than two years of age, if you're less than 550 grams, you will not need chemotherapy because that's not accurate based on your biology studies. You will have to add chemotherapy if you have loss of heterozygosity at 1p16Q. The Children's Oncology Group updates stated that adverse biologic factors are associated with worse prognosis in stage two patients, but not in stage one favorable history of Wilm's tumor patients. But if you have some of the loss of heterozygosity, we're still adding chemotherapy. In summary, there are two major guidelines for treating Wilm's tumor, leading to differences in treatment decisions worldwide. The North American Children's Oncology Group approach, which often involves immediate surgery, and the European Society of Pediatric Oncology approach, which typically starts with chemotherapy before surgery. Genetic markers like loss of heterozygosity at specific chromosomes can indicate a higher risk of the cancer recurrence and guide decisions about adding chemotherapy treatments. Global Cast MD along with Cincinnati Children's Hospital, sharing knowledge to improve child health around the globe.