Peter Ehrlich

385 statements · 5 topics

Congenital Lung Lesions (CPAM) · guest expert Neuroblastoma · guest expert Sarcoma (Ewing/Rhabdo) · guest expert Soft Tissue Sarcoma (lymph nodes) · guest expert Wilms Tumor · guest expert

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There 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.

quote · Congenital Lung Lesions (CPAM)

When 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

quote · Congenital Lung Lesions (CPAM)

Fifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant.

quote · Neuroblastoma

Just because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor

quote · Neuroblastoma

The main factor that predicts outcome in stage 3 is whether they're lymph node positive or not.

quote · Sarcoma (Ewing/Rhabdo)

Preoperative 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.

guideline · Sarcoma (Ewing/Rhabdo)

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Peter's statements about Congenital Lung Lesions (CPAM) 55 statements

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Wilms Tumor: Audio Chapter

Quotation 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. Open

Statement The 'claw sign' on CT—normal kidney displaced into a horseshoe pattern around a mass—is a classic feature of Wilms tumor. Open

Statement Wilms tumor tends to push structures out of the way rather than growing into them, whereas neuroblastoma grows around structures like blood vessels. Open

Quotation In 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. Open

Statement In North America, the preferred approach for resectable Wilms tumor is primary nephrectomy (total nephrectomy and ureterectomy with lymph node sampling). Open

Statement Preoperative 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. Open

Statement If 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. Open

Quotation There'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 Open

Statement Biopsy with residual gross tumor has always been treated as stage 3 abdominal disease, requiring three-drug chemotherapy plus flank radiation. Open

Statement Evidence suggests higher risk of tumor rupture when tumors reach 13-15 centimeters or larger, which may warrant consideration of preoperative chemotherapy. Open

Statement For 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. Open

Statement COG 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. Open

Statement COG 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. Open

Statement COG 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. Open

Statement COG stage 4 is hematogenous metastasis to lung, liver (most common), bone, or brain. Stage 5 is bilateral renal tumor involvement. Open

Statement Stage 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. Open

Statement If 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). Open

Quotation Just because they have a lung lesion doesn't mean that you shouldn't take out the primary tumor Open

Statement Fifteen percent of girls who receive pulmonary radiation for Wilms tumor develop breast cancer, a significant late effect. Open

Quotation Fifteen percent of the girls who get pulmonary radiation from Wilms tumor end up getting breast cancer, which is significant. Open

Statement Recent 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. Open

Quotation There 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. Open

Quotation About 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. Open

Statement For 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. Open

Statement SIOP 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). Open

Statement In 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. Open

Quotation The main factor that predicts outcome in stage 3 is whether they're lymph node positive or not. Open

Statement The 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. Open

Statement Outcomes for stage 1 and stage 2 patients are basically identical between COG and SIOP treatment approaches. Open

Quotation When 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. Open

Statement Some Wilms tumor patients present with low hemoglobin because the tumor ruptures internally and bleeds. Open

Quotation There 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. Open

Statement Acquired 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. Open

Statement Right-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. Open

Quotation These 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. Open

Statement Wilms 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. Open

Statement A 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. Open

Quotation When 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 Open

Statement IVC tumor extension is not a negative prognostic factor if the tumor is completely resected. Open

Quotation The major complication rate in patients goes up. Those include mortalities, number of blood transfusions, ICU stay, complications go up Open

Statement Major 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. Open

Quotation Todd, 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. Open

Statement Favorable histology Wilms tumor has three components: blastema, stroma, and epithelial (triphasic tumor). Tumors with only two components are also considered favorable histology. Open

Statement Patients 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. Open

Statement Patients 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. Open

Statement Unfavorable histology is classified as focal anaplasia or diffuse anaplasia based on number of high-power fields showing anaplasia, with different treatments for each. Open

Statement Renal cell carcinoma in children has no good therapy, particularly for metastatic disease. Open

Statement Clear 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. Open

Statement Bilateral Wilms tumors occur in 8-10% of all children with Wilms tumor. Open

Statement The 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. Open

Statement Event-free survival for bilateral Wilms tumor on NWTS-5 was 61% compared to 88% for unilateral tumors; overall survival was 80% vs. 95%. Open

Statement Maximum tumor response to chemotherapy in most children with Wilms tumor occurs by 12 weeks, and early response at 6 weeks predicts late response. Open

Statement In 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. Open

Statement Biopsy 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. Open

Statement If biopsying bilateral tumors, both kidneys should be biopsied because there is discordant pathology in up to 20% of patients. Open

Peter's statements about Neuroblastoma 110 statements

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