The classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.
The classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.
The classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.
The classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.
The classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.
The classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 24 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 24 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 24 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 24 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 24 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 24 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 24 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 24 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 24 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 24 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 24 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 24 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 24 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 24 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 24 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 24 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 24 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 24 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 24 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 24 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 24 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 24 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 24 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 24 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 24 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 24 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 24 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 24 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 24 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 24 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 24 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 24 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 24 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 24 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 24 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 24 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 24 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 24 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 24 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 24 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 24 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 24 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 24 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 24 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 24 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 24 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 24 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 24 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 24 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 24 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 24 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 24 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 24 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 24 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 8 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 8 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 8 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 8 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 8 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 8 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 8 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 8 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 8 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 8 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 8 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 8 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 8 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 8 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 8 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 8 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 8 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 8 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 8 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 8 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 8 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 8 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 8 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 8 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 8 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 8 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 8 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 8 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 8 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 8 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 8 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 8 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 8 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 8 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 8 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 8 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 8 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 8 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 8 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 8 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 8 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 8 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 8 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 8 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 8 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 8 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 8 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 8 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 8 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 8 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 8 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 8 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 8 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 8 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 8 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 8 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 8 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 8 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 8 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 8 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 8 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 8 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 8 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 8 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 8 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 8 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 8 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 8 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 8 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 8 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 8 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 8 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 8 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 8 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 8 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 8 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 8 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 8 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 8 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 8 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 8 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 8 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 8 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 8 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 8 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 8 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 8 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 8 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 8 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 8 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 8 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 8 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 8 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 8 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 8 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 8 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 8 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 8 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 8 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 8 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 8 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 8 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 8 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 8 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 8 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 8 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 8 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 8 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
▶Ep 8 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 12 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 12 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 12 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 12 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 12 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 12 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 12 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 12 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 12 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 12 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 12 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 12 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 12 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 12 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 12 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 12 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 12 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 12 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 12 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 12 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 12 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 12 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 12 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 12 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 12 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 12 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 12 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 12 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 12 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 12 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 12 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 12 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 12 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 12 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 12 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 12 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 12 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 12 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 12 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 12 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 12 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 12 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 12 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 12 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 12 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 12 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 12 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 12 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 12 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 12 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 12 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 12 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 12 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 12 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 9 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 9 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 9 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 9 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 9 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 9 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 9 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 9 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 9 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 9 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 9 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 9 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 9 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 9 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 9 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 9 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 9 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 9 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 9 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 9 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 9 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 9 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 9 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 9 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 9 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 9 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 9 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 9 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 9 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 9 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 9 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 9 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 9 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 9 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 9 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 9 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 9 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 9 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 9 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 9 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 9 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 9 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 9 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 9 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 9 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 9 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 9 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 9 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 9 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 9 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 9 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 9 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 9 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 9 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 2 · 6:23
guidelineIn North America, the preferred approach for most renal tumors is primary total nephrectomy with ureterectomy and lymph node sampling↗
▶Ep 2 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into IVC beyond the infrahepatic level, if tumor is so large it impairs respiratory status, if major liver or bowel resection would be required, or if only one functioning kidney exists↗
▶Ep 2 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 2 · 12:19
quoteIf you just often Wilmston which will push up to the liver and some people will take a little rim of that just to make sure they have a clear margin, that's completely different.↗
▶Ep 2 · 14:33
clinicalTumors between 13–15 centimeters or larger have higher risk of rupture and may warrant consideration of preoperative chemotherapy↗
▶Ep 2 · 15:55
guidelineBiopsy is recommended when giving preoperative chemotherapy because imaging cannot distinguish between Wilms tumor, rhabdoid tumor, clear cell sarcoma, or determine favorable vs unfavorable histology↗
▶Ep 2 · 17:33
clinicalCore needle biopsy requires at least 10 cores, with accuracy increasing between 10 and 20 cores; fine needle aspiration cannot diagnose anaplasia↗
▶Ep 2 · 20:00
guidelineStage 1 is tumor limited to kidney, completely resected, no capsular invasion, vessels not involved, negative margins and lymph nodes↗
▶Ep 2 · 20:28
guidelineStage 2 is completely resected tumor with regional extension such as capsular penetration or renal sinus invasion, but negative lymph nodes and margins↗
▶Ep 2 · 21:00
guidelineStage 3 includes biopsied tumors with gross residual, positive lymph nodes, peritoneal penetration, positive margins, microscopic residual from spillage, or piecemeal removal↗
▶Ep 2 · 21:58
guidelineStage 4 is hematogenous metastasis to lung, liver, bone, or brain; stage 5 is bilateral renal involvement↗
▶Ep 2 · 23:08
guidelineStage 1 or 2 abdominal disease without lung metastases receives only two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation↗
▶Ep 2 · 23:37
clinicalThe main late effects of concern are renal failure, second malignancies, pregnancy problems, hypertension, and cardiovascular disease, primarily driven by radiation and doxorubicin exposure↗
▶Ep 2 · 24:15
guidelineIf a patient has lung metastases but stage 1 or 2 abdominal disease after primary nephrectomy, they avoid abdominal radiation even though they receive three-drug chemotherapy for the lung disease↗
▶Ep 2 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer↗
▶Ep 2 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 2 · 26:24
clinicalIn COG protocols, approximately 40% of patients with pulmonary metastases who achieve complete response by 6 weeks can avoid pulmonary radiation without compromising survival↗
▶Ep 2 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with response assessment at 4 and 8 weeks before surgery↗
▶Ep 2 · 31:19
guidelineSIOP classifies patients post-treatment as low risk (complete necrosis), intermediate risk (based on blastema percentage), or high risk (predominantly blastema or anaplasia)↗
▶Ep 2 · 32:45
clinicalIn stage 3 disease, the main prognostic factor is lymph node status, followed by loss of heterozygosity at 1p and 16q, and 1q gain↗
▶Ep 2 · 33:34
clinicalAttempting resection and having to bail out with biopsy does not worsen prognosis compared to empiric biopsy for stage 3 disease↗
▶Ep 2 · 35:11
clinicalSome Wilms tumor patients develop acquired von Willebrand disease; while usually clinically insignificant, rare cases have had significant intraoperative bleeding↗
▶Ep 2 · 36:25
clinicalRight-sided tumors can distort anatomy leading to potential duodenal injury, superior mesenteric artery injury, or IVC injury↗
▶Ep 2 · 37:19
clinicalThe classic Wilms tumor surgical approach involves transverse or subcostal incision, mobilizing the kidney onto its pedicle, then identifying and dividing the ureter distally, followed by hilar vessel control↗
▶Ep 2 · 39:23
clinicalAdrenalectomy is not necessary; there are no reports of adrenal insufficiency and adrenal vein tumor presence does not correlate with outcomes↗
▶Ep 2 · 39:49
guidelineLymph node sampling should aim for at least 5–6 nodes from the renal hilum and para-aortic or paracaval regions↗
▶Ep 2 · 42:31
clinicalTaking a rim of diaphragm or small piece of liver to avoid violating tumor capsule does not upstage the tumor if the tumor itself is not divided↗
▶Ep 2 · 43:32
clinicalVery low-risk patients (less than 2 years old, tumor less than 550 grams, stage 1, favorable histology) can be treated with surgery alone with greater than 95% survival↗
▶Ep 2 · 45:01
clinicalThe 10% of very low-risk patients who relapse after surgery alone have 100% salvage survival with chemotherapy↗
▶Ep 2 · 46:14
clinicalIVC tumor extension is not a negative prognostic factor if the tumor can be completely resected↗
▶Ep 2 · 47:07
clinicalFor infrahepatic IVC thrombus, the ideal technique is to mobilize the kidney, ligate the renal artery, make a small nick in the renal vein, and slide the thrombus out in one piece↗
▶Ep 2 · 47:43
clinicalMajor complication rates including mortality, transfusions, and ICU stay increase significantly when IVC thrombus extends above the infrahepatic level↗
▶Ep 2 · 48:49
epidemiologicalIn the largest series of IVC thrombus extending beyond infrahepatic cava treated with primary surgery, there was 26–30% major morbidity and some mortality↗
▶Ep 2 · 49:47
clinicalIntraoperative ultrasound is used to assess the superior extent of IVC thrombus and determine if partial or complete caval occlusion is needed for resection↗
▶Ep 2 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 2 · 52:07
quoteThree months ago I had to take out a kit, and they had all collaterals and the easiest thing I did, I Took out the whole cava because he wasn't using it↗
▶Ep 2 · 54:41
clinicalLoss of heterozygosity at both 1p and 16q occurs in 5–7% of patients and is associated with 10% worse survival in stage 1–2 and 18% worse in stage 3–4↗
▶Ep 2 · 55:23
guidelinePatients with loss of heterozygosity at 1p and 16q receive intensified treatment: stage 1–2 get three drugs instead of two, stage 3–4 get five-drug regimen M↗
▶Ep 2 · 55:58
clinicalUnfavorable histology is classified as focal or diffuse anaplasia based on the number of high-power fields showing anaplastic features↗
▶Ep 2 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes particularly for low stages, while rhabdoid tumors have terrible outcomes except for stage 1↗
▶Ep 2 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8–10% of all Wilms tumor cases↗
▶Ep 2 · 58:07
guidelineThe strategy for bilateral Wilms is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy on at least one kidney, avoiding dialysis↗
▶Ep 2 · 58:51
epidemiologicalHistorical outcomes for bilateral Wilms tumor were significantly worse than unilateral: 61% event-free survival and 80% overall survival compared to 88% and 95% respectively↗
▶Ep 2 · 59:19
guidelineThe COG bilateral Wilms study used VAD induction chemotherapy with response assessment at 6 and 12 weeks, as maximum response typically occurs by 12 weeks↗
▶Ep 2 · 1:00:29
clinicalIn typical bilateral Wilms presentations (under 36 months, classic imaging), biopsy is not required as it is almost universally Wilms tumor; only 1 of 250 enrolled patients had rhabdoid tumor↗
▶Ep 2 · 1:01:56
guidelineBiopsy is recommended for atypical bilateral presentations: older children (8–10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely↗
▶Ep 2 · 1:02:14
clinicalWhen biopsying bilateral disease, both kidneys should be biopsied as there is discordant pathology in up to 20% of cases↗
Wilms Tumor: Audio Chapter
▶Ep 10 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 10 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 10 · 3:49
clinicalThe 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor.↗
▶Ep 10 · 3:49
quoteThe classic feature is something called a sign where you have the kidney. The normal kidney being displaced into a horseshoe pattern and fitting inside that horseshoe pattern is a tumor and so it looks like the normal kidney is grabbing the mass that's coming out of it.↗
▶Ep 10 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 10 · 4:16
clinicalWilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels.↗
▶Ep 10 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 10 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 10 · 6:23
quoteIn North America we believe and prefer that the next step in the treatment of children with renal tumors is to go ahead and perform surgery.↗
▶Ep 10 · 6:23
guidelineIn North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling).↗
▶Ep 10 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 10 · 7:01
guidelinePreoperative chemotherapy is recommended if tumor extends into the IVC beyond the infrahepatic vena cava, if tumor is so large it compromises respiratory status, if major liver or bowel resection would be required, if only one functioning kidney exists, or if bilateral tumors are present.↗
▶Ep 10 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 10 · 10:24
clinicalIf a tumor requires major liver or bowel resection, biopsy and preoperative chemotherapy are preferred because complication rates are higher when these organs are resected at the same time as the kidney, and most tumors will respond to chemotherapy.↗
▶Ep 10 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 10 · 10:48
quoteThere's no reason to do that and and overall the consequence in that case of of upstaging, where does that so it's not really upstaging↗
▶Ep 10 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 10 · 11:18
guidelineBiopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation.↗
▶Ep 10 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 10 · 14:33
clinicalEvidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy.↗
▶Ep 10 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 10 · 17:19
guidelineFor biopsy, open biopsy provides large tissue sections; large-core needle biopsy (10-20 cores) has proven accurate in several series. Fine needle aspiration or single-pass true-cut biopsy cannot diagnose anaplasia and should not be used.↗
▶Ep 10 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 10 · 20:00
guidelineCOG stage 1 is tumor limited to kidney, completely resected, no capsular invasion, no rupture or biopsy, vessels of renal sinus not involved, negative margins, and negative regional lymph nodes.↗
▶Ep 10 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 10 · 20:28
guidelineCOG stage 2 is complete resection with negative margins but tumor extends beyond kidney through capsular penetration, renal sinus invasion, or blood vessel involvement within the nephrectomy specimen outside the primary kidney.↗
▶Ep 10 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 10 · 21:00
guidelineCOG stage 3 includes: biopsy with gross residual tumor, positive lymph nodes, peritoneal surface penetration with implants, positive margins, microscopic residual from intraoperative spill, incomplete resection, piecemeal removal, or renal vein division with tumor present.↗
▶Ep 10 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 10 · 21:58
guidelineCOG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement.↗
▶Ep 10 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 10 · 23:08
guidelineStage 1-2 abdominal disease without lung metastases receives two-drug chemotherapy (vincristine and dactinomycin) for shorter duration without abdominal radiation, significantly reducing late effects including renal failure, second malignancies, and cardiovascular disease.↗
▶Ep 10 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 10 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 10 · 24:15
clinicalIf a patient has a lung lesion but stage 1-2 abdominal disease after primary nephrectomy with negative lymph nodes, the child avoids abdominal radiation (though still receives chemotherapy for the lung lesion).↗
▶Ep 10 · 24:15
quoteJust because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor↗
▶Ep 10 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 10 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 10 · 26:08
quoteFifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.↗
▶Ep 10 · 26:08
epidemiologicalFifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect.↗
▶Ep 10 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 10 · 26:24
clinicalRecent COG data shows approximately 40% of patients with pulmonary metastases who have complete response at 6 weeks can avoid pulmonary radiation without affecting event-free or overall survival, with 80-85% not relapsing.↗
▶Ep 10 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 10 · 26:44
quoteThere was about 40% of the patients, they were not given pulmonary radiation and looking at that group at this point in time looking at relapses, there was about 80% to 85% that did not relapse.↗
▶Ep 10 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 10 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 10 · 28:09
clinicalFor a single lung lesion remaining at 6 weeks that did not respond to chemotherapy, thoracoscopic biopsy is reasonable because 50-60% of such lesions may not be cancer (could be scar), and if benign, the patient would not need pulmonary radiation.↗
▶Ep 10 · 28:09
quoteAbout 50 to 60% of the time those lesions may not turn out to be cancer. They could be scarred. They could be a variety of things.↗
▶Ep 10 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 10 · 30:16
guidelineSIOP protocols use preoperative chemotherapy for all patients at higher doses than COG, with evaluation at 4 and 8 weeks, then resection followed by post-chemotherapy pathology-based risk stratification (low, intermediate, high risk).↗
▶Ep 10 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 10 · 31:19
clinicalIn SIOP protocols, predominantly blastema component after preoperative chemotherapy is a negative prognostic factor, but blastema percentage does not correlate with outcome in COG primary nephrectomy patients.↗
▶Ep 10 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 10 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 10 · 32:50
quoteThe main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.↗
▶Ep 10 · 32:50
clinicalThe main factor predicting outcome in stage 3 disease is whether lymph nodes are positive, along with genetic changes including loss of heterozygosity and 1Q gain.↗
▶Ep 10 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 10 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 10 · 33:58
quoteWhen you look at the outcomes for stage 1 and stage 2 patients between the children's oncology group and the psyop groups, they're basically identical.↗
▶Ep 10 · 33:58
clinicalOutcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches.↗
▶Ep 10 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 10 · 34:46
clinicalSome Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds.↗
▶Ep 10 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 10 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 10 · 35:11
quoteThere is something called acquired von Willebrand's disease which these patients get. In the majority of cases it is meaningless, but there have been reports in a few case series where these patients may bleed a lot.↗
▶Ep 10 · 35:11
clinicalAcquired von Willebrand disease occurs in Wilms tumor patients; in most cases it is meaningless, but a few case series report significant intraoperative bleeding until tumor removal.↗
▶Ep 10 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 10 · 36:25
clinicalRight-sided tumors can cause complications including adrenal vein injury, duodenal injury (due to proximity), superior mesenteric artery injury, and IVC injury due to anatomic distortion.↗
▶Ep 10 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 10 · 38:20
quoteThese tumors tend to be large and although ideally you would like to identify the renal artery and the renal vein, it's been well recognized. It's even back in Gross's book that these, if you can't do that, then you shouldn't try and do it up front.↗
▶Ep 10 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 10 · 42:31
clinicalWilms tumors can cause intense inflammatory reaction making them adherent to diaphragm or liver; taking a rim of diaphragm or liver capsule to avoid tumor violation does not upstage the tumor if the tumor itself is not divided.↗
▶Ep 10 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 10 · 43:32
clinicalA small subset of very young patients (<24 months) with stage 1 favorable histology tumors <550g can be treated with surgery alone without chemotherapy, with >95% overall survival. Those who relapse (about 10%) have 100% salvage with chemotherapy.↗
▶Ep 10 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 10 · 46:14
quoteWhen you look at the outcomes of patients with tumor that extends into the cava or even up to the atrium, that's not a negative prognostic factor↗
▶Ep 10 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 10 · 46:27
clinicalIVC tumor extension is not a negative prognostic factor if the tumor is completely resected.↗
▶Ep 10 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 10 · 47:43
quoteThe major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up↗
▶Ep 10 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 10 · 48:49
clinicalMajor complication rate for primary surgery with tumor extending beyond infrahepatic IVC is 26-30%, including mortality, increased transfusions, and ICU stay, supporting preoperative chemotherapy for these cases.↗
▶Ep 10 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 10 · 51:28
quoteTodd, I, I've read 6000 operative notes, OK. The amount of time that somebody actually had it go all the way up there like that and stop at the infrapatic cava, I don't even recall reading a case where that happened.↗
▶Ep 10 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 10 · 52:39
clinicalFavorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology.↗
▶Ep 10 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 10 · 54:41
clinicalPatients with loss of heterozygosity of both 1p and 16q (5-7% of patients) have significantly worse outcomes regardless of stage. Stage 1-2 patients with LOH have about 10% lower overall survival; stage 3-4 patients have 18% lower survival.↗
▶Ep 10 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 10 · 55:07
guidelinePatients with both 1p and 16q loss of heterozygosity receive augmented therapy: doxorubicin is added for stage 1-2 disease, and five-drug regimen M is used for stage 3-4 disease.↗
▶Ep 10 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 10 · 55:58
clinicalUnfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each.↗
▶Ep 10 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 10 · 56:51
clinicalRenal cell carcinoma in children has no good therapy, particularly for metastatic disease.↗
▶Ep 10 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 10 · 57:01
clinicalClear cell sarcoma of the kidney has reasonable treatment outcomes, particularly for low stages. Rhabdoid tumors have poor outcomes except for stage 1; most present at stage 3-4 with terrible outcomes.↗
▶Ep 10 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 10 · 57:46
epidemiologicalBilateral Wilms tumors occur in 8-10% of all children with Wilms tumor.↗
▶Ep 10 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 10 · 58:07
guidelineThe strategy for bilateral Wilms tumor is preoperative chemotherapy to shrink tumors enough to allow partial nephrectomy of at least one kidney (ideally both) to avoid dialysis.↗
▶Ep 10 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 10 · 58:40
epidemiologicalEvent-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%.↗
▶Ep 10 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 10 · 59:37
clinicalMaximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response.↗
▶Ep 10 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 10 · 1:00:31
epidemiologicalIn children presenting under 36 months with bilateral renal tumors, it is almost universally Wilms tumor. In a recent COG study of 250 enrolled patients, only one turned out to have rhabdoid tumor.↗
▶Ep 10 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 10 · 1:01:56
guidelineBiopsy is recommended for bilateral tumors in older patients (8-10 years) or those with syndromes like von Hippel-Lindau where renal cell carcinoma is more likely.↗
▶Ep 10 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
▶Ep 10 · 1:02:16
clinicalIf biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients.↗
