# Topics in 10: Wilms Tumor — GCMD Library

<p>Drs Andrew Davidoff, Todd Ponsky and Rae Hanke come together to provide you the essentials on the diagnosis and management of Wilms Tumor.<p>Intro and outro tracks are adapted from "I dunno" by grapes, featuring J Lang, Morusque. Artist URL: ccmixter.org/files/grapes/16626<br></p></p><p><a href="http://videolibrary.globalcastmd.com/topics-in-10-wilms-tumor"></a></p><h2><ul><li><b>What is Wilms tumor</b>?</li></ul></h2><ul><ul><li>Second most common intra-abdominal tumor in children</li><li>5th most common tumor in children overall</li><li>75% of tumors are found in children < 5 years</li><li>Peak incidence at 2-3 years old</li><li>Survival is >90% overall</li><ul><li>Affected by histology</li><li>Categorized as favorable or unfavorable (Anaplastic)</li><li>Anaplastic is unfavorable, found in about 10% of cases, but causes over 50% of mortality from Wilm’s tumor</li></ul></ul></ul><h2><ul><li><b>How does it present</b>?</li></ul></h2><ul><ul><li>Asymptomatic abdominal mass</li><li>Other signs/symptoms (seen in about 25%)</li><li>Malaise, abdominal pain, hypertension, microscopic or gross hematuria</li></ul></ul><h2><ul><li><b>What do you do to work up suspected renal mass</b>?</li></ul></h2><ul><ul><li>Start with an abdominal ultrasound</li><li>CT abdomen/pelvis is the definitive imaging modality</li><ul><li>Confirm solid renal mass</li><li>Ensure there are two kidneys</li><li>Rule out synchronous bilateral disease</li><li>Evaluate for intravascular extension (found in 6%)</li></ul><li>Consider echocardiogram if intracardiac thrombus is suspected</li><li>Chest CT to evaluate for lung metastasis (most common site)</li></ul><li><b>Staging</b>: surgical pathological staging determined by the Children’s Oncology Group (COG)</li><ul><li>Stage 1: Localized Wilm’s confined to the renal capsule</li><li>Stage 2: Penetrate renal capsule, but have been resected with negative margins</li><li>Stage 3: Biopsy or rupture (pre-operative or intra-operative), positive margins (residual disease), lymph node involvement, or use of preop chemo</li><li>Stage 4: metastasis (12% of patients)</li><ul><li>Determine local stage—dictates whether you should use abdominal radiation or not and to what field</li></ul><li>Stage 5: synchronous bilateral tumors</li><ul><li>Need to determine local stage for each side</li></ul></ul></ul><h2><ul><li><b>Treatment</b></li></ul></h2><ul><ul><li><b>Unilateral disease</b>:</li><ul><li>Surgery: Remove primary tumor by Radical nephrectomy with regional lymph node sampling</li><ul><li>Most are resectable at presentation</li><li>Node sampling: Critical!</li><ul><li>Absence of abnormal nodes on preoperative imaging or intraoperative inspection are NOT predictive</li><li>Failure to sample automatically upstages disease to Stage 3</li><li>Nodal metastasis is associated with likelihood of relapse and poorer prognosis</li><li>Failure to operate before neoadjuvant chemotherapy automatically upstages disease to Stage 3 [potentially inducing long-term toxicity unnecessarily]</li></ul><li>No partial nephrectomy or laparoscopic nephrectomy in unilateral non-syndromic disease UNLESS you are a part of a clinical trial</li></ul><li>Chemotherapy or Radiation?</li><ul><li>Stage 1 & 2 (favorable staging): Adjuvant chemotherapy is limited to Vincristine and Actinomycin B</li><li>Stage 1 with tumor/kidney <550 grams and age <2 yrs: don’t require adjuvant chemotherapy!</li><li>Research being conducted to potentially raise this age limit and weight!</li><li>Stage 3 & 4: (favorable staging): Stage 1 & 2 chemotherapy PLUS Doxorubicin with flank irradiation</li><li>Anaplastic histology:</li><ul><li>Significantly worse outcome and require more intense chemotherapy</li><li>Focal and diffuse classifications influence specific neoadjuvant therapy</li></ul></ul></ul><li><b>Bilateral Wilms tumor</b> = Stage 5</li><ul><li>Bilateral synchronous disease found in 5% of patients  </li><li>Increased risk of renal failure</li><li>Biopsy not required in bilateral solid renal masses because diagnosis is very likely Wilm’s tumor</li><ul><li>Studies show the biopsy rarely shows dysplasia if it does exist</li><li>Biopsy in bilateral disease doesn’t upstage the disease like it does in unilateral disease</li></ul><li>Neoadjuvant chemotherapy: to shrink tumor prior to surgery—preserve normal renal function</li><ul><li>Vincristine, Actinomycin B, and Doxorubicin: Similar to Stage 3 & 4 favorable histology adjuvant regimen</li></ul><li>Surgery:</li><ul><li>Consider bilateral nephron sparing surgery in all bilateral tumors</li><li>Operate 6-12 weeks after neoadjuvant chemotherapy (anything longer is discouraged)</li></ul></ul><li><b>Solitary</b> <b>kidney</b>: similar to bilateral synchronous chemotherapy regimen</li><ul><li>Unilateral tumor but predisposed to developing second tumor</li><li>WAGER or Beckwith-Weidemann syndrome</li><li>Chemotherapy regimen usually doesn’t include Doxorubicin</li><li>Radiation isn’t mandated like it is in unilateral Wilm’s tumor**</li></ul><li><b>Intravascular tumor extension</b></li><ul><li>Challenging</li><li>Determined by preoperative imaging and confirmed intraoperatively</li><li>Management depends on extent:</li><ul><li>Renal vein and proximal IVC: remove enbloc with kidney tumor</li><li>Further into IVC but below hepatic veins: can be withdrawn after obtaining proximal and distal control</li><li>Above hepatic veins/into right atrium:</li><ul><li>Has higher rate of morbidity</li><li>Need neoadjuvant chemotherapy</li><li>If it persists after chemotherapy, likely requires cardiopulmonary bypass to remove the extent of disease</li></ul></ul></ul><li><b>Metastasis</b></li><ul><li>Found in 12% of patients at diagnosis</li><li>Spread hematogenously, with 80% metastasis found in lungs</li><li>Response-based chemotherapy approach:</li><ul><li>Give three drug chemotherapy regimen and repeat imaging at week six</li><li>Rapid responders: Disappears radiographically or biopsy shows noviable tumor in residual nodules</li><ul><li>Continue on chemotherapy but don’t need pulmonary radiation</li></ul><li>Slow or incomplete responders: no complete resolution at 6 weeks</li><ul><li>More intense chemotherapy and whole lung radiation</li></ul></ul></ul></ul></ul><h2><ul><li><b>Wilms Tumor Clinical Pearls</b>:</li></ul></h2><ul><ul><li>Most common renal tumor in children</li><li>Outstanding overall survival at greater than 90%</li><li>Worse with anaplastic histology</li><li>Management/Treatment:</li><br>Unilateral disease:<br><li>Radical nephrectomy with lymph node sampling, followed by vincristine and actinomycin B (STAGE 1-2)</li><li>Stage 3: additional doxorubicin and radiation therapy</li><br>Bilateral disease:<br><li>Neoadjuvant three drug therapy and then nephron sparing surgery if possible</li><br>Intravascular involvement: approach based on extent of tumor<br><li>Above hepatic veins—neoadjuvant chemotherapy</li><br>Lung metastasis:<br><li>Management is now response-based to avoid pulmonary radiation</li><br><br><b>Additional reading resources:</b><br><li>















<p><a href="http://bit.ly/2DUIc3M">http://bit.ly/2DUIc3M</a></p>

</li><li><p><b><a href="http://bit.ly/36cf9F4">http://bit.ly/36cf9F4</a></b></p></li><li><p><b>Link to full length podcast on Wilms Tumor:</b> <a href="http://bit.ly/2Rt6UjS">http://bit.ly/2Rt6UjS</a></p></li></ul></ul><p></p><p>Intro and outro tracks are adapted from "I dunno" by grapes, featuring J Lang, Morusque. Artist URL: <a href="http://bit.ly/38oiDGq">http://bit.ly/38oiDGq</a></p>

Type: podcast · 12 min · posted 2020-12-30
Canonical: https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583

## Chapters
- [0:00](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=0) Introduction and Epidemiology
- [1:17](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=77) Clinical Presentation and Diagnostic Workup
- [2:55](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=175) Staging System
- [4:03](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=243) Treatment of Unilateral Disease
- [6:49](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=409) Management of Bilateral Disease
- [8:37](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=517) Intravascular Tumor Extension
- [9:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=593) Metastatic Disease Management
- [11:03](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=663) Summary and Conclusion

## Statements
- "Wilms tumor is the second most common intra-abdominal tumor in children and fifth most common tumor in children overall" — Rae Hanke (epidemiological) [0:00](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=0)
- "Approximately 75% of Wilms tumor cases occur in children younger than 5 years of age, with peak incidence at 2 to 3 years of age" — Andrew Davidoff (epidemiological) [0:28](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=28)
- "Survival for patients with Wilms tumor when considered as a whole is currently greater than 90%" — Andrew Davidoff (epidemiological) [0:28](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=28)
- "Anaplastic histology comprises only about 10% of Wilms tumor cases but contributes to over 50% of Wilms tumor mortality" — Andrew Davidoff (epidemiological) [0:28](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=28)
- "Children with Wilms tumor typically present with an asymptomatic abdominal mass" — Andrew Davidoff (clinical) [1:20](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=80)
- "Associated signs and symptoms such as malaise, pain, microscopic or gross hematuria are found in only about 25% of children with Wilms tumor, as is hypertension" — Andrew Davidoff (clinical) [1:20](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=80)
- "The workup of a child with suspected Wilms tumor usually begins with ultrasound" — Andrew Davidoff (guideline) [1:46](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=106)
- "CT of the abdomen and pelvis is generally the definitive imaging study of choice for patients suspected of having a renal tumor based on ultrasound" — Andrew Davidoff (guideline) [1:46](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=106)
- "Intravascular tumor extension occurs in about 6% of Wilms tumor cases" — Andrew Davidoff (epidemiological) [1:46](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=106)
- "The most common site of metastatic spread of Wilms tumor is the lungs" — Andrew Davidoff (clinical) [2:44](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=164)
- "A chest CT should be included in the initial evaluation of a child suspected of having a Wilms tumor" — Andrew Davidoff (guideline) [2:44](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=164)
- "Localized Wilms tumors that are confined within the renal capsule are stage 1" — Andrew Davidoff (guideline) [2:59](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=179)
- "Wilms tumors that penetrate the renal capsule but are resected with negative margins are stage 2" — Andrew Davidoff (guideline) [2:59](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=179)
- "Stage 3 Wilms tumor includes circumstances such as biopsy or rupture (preoperative or intraoperative), positive resection margin or gross residual disease, lymph node involvement, or administration of preoperative chemotherapy" — Andrew Davidoff (guideline) [2:59](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=179)
- "Metastatic disease occurs in about 12% of Wilms tumor patients and is considered stage 4" — Andrew Davidoff (epidemiological) [2:59](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=179)
- "Patients with synchronous bilateral Wilms tumor are stage 5" — Andrew Davidoff (guideline) [2:59](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=179)
- "For unilateral tumors, upfront resection with regional lymph node sampling is currently the recommendation from the Children's Oncology Group" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "Most Wilms tumors are resectable at presentation because even large tumors rarely invade surrounding structures" — Andrew Davidoff (clinical) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "Failure to perform upfront resection and instead administering neoadjuvant chemotherapy in COG results in classification as stage 3, mandating flank irradiation and doxorubicin, each associated with significant long-term toxicities" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "Treatment of favorable histology Wilms tumor stage 1 or 2 is limited to vincristine and actinomycin D" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "In rare circumstances when tumor is stage 1, weighs less than 550 grams (tumor plus kidney), and patient is less than 2 years of age, no adjuvant chemotherapy is given" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "The presence of nodal involvement is associated with an increased incidence of tumor relapse and a poorer prognosis" — Andrew Davidoff (clinical) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "Lymph node sampling should be performed even in the absence of abnormal nodes on preoperative imaging or gross inspection during operative exploration, since these circumstances don't reliably predict lymph node negativity" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "Partial nephrectomy for patients with unilateral non-syndromic disease and laparoscopic nephrectomy are not currently standard of care and should generally only be performed in the context of a clinical trial" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "Anaplastic histology is associated with significantly worse outcome and is treated with more intensive chemotherapy" — Andrew Davidoff (clinical) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "A distinction is made between focal and diffuse anaplasia when determining specific adjuvant therapy" — Andrew Davidoff (guideline) [4:06](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=246)
- "About 5% of children with Wilms tumor will present with synchronous bilateral disease or stage 5 disease" — Andrew Davidoff (epidemiological) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "Patients with bilateral Wilms tumor receive neoadjuvant chemotherapy with three drugs to shrink tumors prior to surgery and facilitate preservation of normal renal parenchyma due to increased risk of renal failure" — Andrew Davidoff (guideline) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "A biopsy is not required in children with bilateral solid renal masses as bilateral Wilms tumor is the very likely diagnosis" — Andrew Davidoff (guideline) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "Biopsies of bilateral renal masses rarely detect anaplasia even when it does exist in the tumor mass" — Andrew Davidoff (clinical) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "A biopsy in bilateral disease doesn't mandate subsequent radiation as it does in patients with unilateral Wilms tumor" — Andrew Davidoff (guideline) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "Bilateral nephron-sparing surgery should be considered in all patients with bilateral Wilms tumor and should be performed after either 6 or 12 weeks of neoadjuvant chemotherapy" — Andrew Davidoff (guideline) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "Longer courses of preoperative chemotherapy than 6 or 12 weeks are definitely discouraged in bilateral Wilms tumor" — Andrew Davidoff (guideline) [6:53](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=413)
- "Tumor extension into the renal vein and proximal inferior vena cava can in most cases be removed en bloc with the kidney and tumor" — Andrew Davidoff (clinical) [8:42](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=522)
- "Primary resection of tumors with extension above the level of the hepatic veins or into the atrium is associated with higher operative morbidity, so neoadjuvant chemotherapy is generally used in these circumstances" — Andrew Davidoff (guideline) [8:42](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=522)
- "Thrombus extending above the hepatic veins that persists after neoadjuvant chemotherapy probably requires cardiopulmonary bypass to safely remove the full extent of disease" — Andrew Davidoff (guideline) [8:42](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=522)
- "About 12% of Wilms tumor patients will have evidence of hematogenous metastases at diagnosis with 80% of these being pulmonary metastases" — Andrew Davidoff (epidemiological) [9:58](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=598)
- "A new response-based approach is being used for patients with stage 4 disease in the Children's Oncology Group" — Andrew Davidoff (guideline) [9:58](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=598)
- "Stage 4 patients treated with three-drug chemotherapy who have radiographic disappearance of lung metastases or tissue confirmation that residual nodules don't contain viable tumor at week 6 are considered rapid responders and won't receive pulmonary irradiation" — Andrew Davidoff (guideline) [9:58](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=598)
- "Patients who don't have complete resolution of pulmonary nodules at 6 weeks are considered slow or incomplete responders, will be switched to more intensive chemotherapy regimen, and will receive whole lung irradiation" — Andrew Davidoff (guideline) [10:47](https://library.globalcastmd.com/watch/topics-in-10-wilms-tumor-1583?t=647)

## Transcript
 It's hard to stay on top of the rapidly changing literature in the limited hours we have in a day. So here at Stay Current, we are starting an ultra-focused podcast series, topics in 10, for those times you need just the cliff notes. Let's get to it. Have you ever asked, What exactly is Wilms' tumor? Wilms' tumor is the second most common intra-abdominal tumor in children, and fifth most common tumor in children overall, and its management is often changing. Dr. Andrew Davidoff, Chairman of Surgery at St. Jude's Children's Research Hospital, joins us to give us the essentials. Approximately 75% of the cases occur in children younger than 5 years of age, with a peak incidence at 2 to 3 years of age. Thankfully, survival for patients with Wilms' tumor, when considered as a whole, is currently greater than 90%. However, a critical prognostic factor that profoundly impacts outcome is histology. This is divided simply into two broad types, favorable and unfavorable, or anaplastic histology. The latter group comprises, thankfully, only about 10% of cases, but does contribute to over 50% of Wilms' tumor mortality. Talk to me about how these children present, typically. Well, interestingly, children with Wilms' tumor typically present with an asymptomatic abdominal mass. Associated signs and symptoms such as malaise, pain, microscopic, or gross hematuria are found in only about 25% of the children, as is hypertension. So, when a child presents with an abdominal mass, what's the typical workup? The workup of a child with an intra-abdominal mass that you suspect of being a Wilms' tumor usually begins with ultrasound. CT of the abdomen and pelvis is generally the definitive imaging study of choice for those patients who are suspected of having a renal tumor based on ultrasound. CT will confirm the presence of a solid renal mass and will also afford the opportunity to visualize the contralateral kidney to confirm its presence and function and to exclude synchronous bilateral disease. Intravascular tumor extension occurs in about 6% of Wilms' tumor cases. Therefore, this should be specifically investigated in preoperative evaluation as it may alter the timing and conduct of surgery. If intracardiac extension of tumor thrombus is suspected, this can be assessed by echocardiography. And where does Wilms metastasize to? The most common site of metastatic spread of Wilms' tumor is the lungs, and so a chest CT should be included in the initial evaluation of a child suspected of having a Wilms' tumor. Can you go over the staging of Wilms' tumor for me? Sure. So the children's oncology group currently uses a surgical pathologic staging system in which localized Wilms' tumors that are confined within the renal capsule are stage 1, while those that penetrate the renal capsule but are resected with negative margins are stage 2. Circumstances that make Wilms' tumor stage 3 are various and include biopsy or rupture, either preoperative or intraoperative, positive resection margin or gross residual disease, lymph node involvement, or the administration of preoperative chemotherapy. Metastatic disease, which occurs in about 12% of patients, is considered stage 4, although the local stage should also be evaluated, as this will determine whether abdominal radiation is indicated and to what field. Patients with synchronous bilateral Wilms' tumor are stage 5, but here again, local stage for each side should still be evaluated. Talk to me about the treatment of a child who's diagnosed with Wilms' tumor. Sure. So, for unilateral tumors, upfront resection with regional lymph node sampling is currently the recommendation from the children's oncology group, and by upfront resection, that's generally radical nephrectomy. And the reason why this approach is favored by the COG is twofold. First, although Wilms' tumors can grow to a large size, even large tumors rarely invade surrounding structures, and so because of this, most Wilms' tumors are resectable at presentation. Secondly, the failure to perform an upfront resection, but instead administering neoadjuvant chemotherapy in the COG, results in the classification of a tumor as stage 3, thus mandating the use of other therapies, including flank irradiation and doxorubicin, each of which is associated with significant long-term toxicities. Treatment of favorable histology Wilms' tumor, that's stage 1 or 2, is just limited to vincristin and actinomycin D. And more recently, in rare circumstances, when the tumor is stage 1, weighs less than 550 grams, that's the tumor weight plus the kidney, and the patient is less than 2 years of age, no adjuvant chemotherapy is given. And actually, raising the age and the weight limits of the tumor plus kidney are currently being considered for study by COG. But it is important that careful lymph node sampling be done, as it's a critical part of any operation for Wilms' tumor, because the presence of nodal involvement is associated with an increased incidence of tumor relapse and a poorer prognosis, although effective, albeit more intensive therapy, exists to treat children with stage 3 disease. So, lymph node sampling should be performed even in the absence of abnormal nodes on preoperative imaging or on gross inspection during operative exploration, since these circumstances really don't reliably predict lymph node negativity. Partial nephrectomy for patients with unilateral, non-syndromic disease, and or laparoscopic nephrectomy are really not currently standard of care and should generally only be performed in the context of a clinical trial. And finally, as I mentioned previously, anaplastic histology is unfortunately associated with a significantly worse outcome and so is treated with more intensive chemotherapy. There is a distinction, though, made between focal and diffuse anaplasia when determining specific adjuvant therapy. Talk to me about a child that presents with bilateral Wilms tumor. Of course. So, about 5% of children with Wilms tumor will present with synchronous bilateral disease or stage 5 disease. And in these circumstances, due to an increased risk of renal failure for these patients, they receive neoadjuvant chemotherapy chemotherapy with three drugs as used for patients with stage 3 or stage 4 favorable histology Wilms tumor in an effort to shrink the tumors prior to surgery and to facilitate the preservation of normal renal parenchyma. Also treated in this manner are patients with Wilms tumor arising in a solitary kidney or, as I mentioned before, those with unilateral Wilms tumor who are at an increased risk for developing metachronous tumor, although these patients usually don't receive doxorubicin as part of their neoadjuvant chemotherapy. A biopsy is not required in children with bilateral solid renal masses as bilateral Wilms tumor is the very likely diagnosis. Although the histologic subtype, favorable or unfavorable, won't be known, studies have shown that biopsies of bilateral renal masses rarely detect anaplasia even when it does exist in the tumor mass. However, a biopsy, if performed, doesn't mandate subsequent radiation as it does in patients with unilateral Wilms tumor. Bilateral nephron-sparing surgery should really be considered in all patients with bilateral Wilms tumor. This should be performed after either 6 or 12 weeks of neoadjuvant chemotherapy. Longer courses of preoperative chemotherapy are definitely discouraged. So, how do you manage a patient that presents with intravascular tumor extension? Yeah, so these cases can be quite challenging. It should be determined by preoperative imaging that there is or isn't intravascular tumor extension. then its presence or absence confirmed intraoperatively. Tumor extension into the renal vein and proximal inferior vena cava can in most cases be removed and blocked with the kidney and tumor. Thrombus that extends further into the vena cava can also be withdrawn from the IVC after gaining proximal and distal control. Proximal control can generally be achieved if the superior extent of the thrombus is below the level of the hepatic veins. However, primary resection of tumors with extension above the level of the hepatic veins or especially into the atrium is associated with higher operative morbidity and so neoadjuvant chemotherapy is generally used in these circumstances. Thrombus that extends above the hepatic veins and which persists to this extent after neoadjuvant chemotherapy probably requires cardiopulmonary bypass to safely remove the full extent of disease. So what if the patient presents with metastases? Yeah, so about 12% of Wilms tumor patients will have evidence of hematogenous metastases at diagnosis with 80% of these being pulmonary metastases. Interestingly, a new response-based approach is being used for the patients with stage 4 disease in the children's oncology group. These patients treated with three-drug chemotherapy who have radiographic disappearance of their lung metastases or who have tissue confirmation that residual nodules don't contain viable tumor at week 6 imaging re-evaluation will be considered rapid responders will continue on this three-drug chemotherapy regimen but won't receive pulmonary irradiation. And patients who don't respond? Patients who don't have complete resolution of pulmonary nodules at 6 weeks will be considered slow or incomplete responders. They will be switched to more intensive chemotherapy regimen and will receive whole lung irradiation. You did it! You just made it through a blitzkrieg on Wilms tumor. So, here's a quick recap of our clinical pearls. Wilms is the most common renal tumor in children. It has an outstanding overall survival at greater than 90% but that's worse with anaplastic histology. The treatment for unilateral disease is a radical nephrectomy with lymph node sampling followed by venchristine and actinomycin B for stages 1 and 2. For stage 3, add doxorubicin and radiation therapy. For bilateral disease, neoadjuvant 3 drug therapy and then nephron sparing surgery, if possible, is the way to go. If there's intravascular involvement, the approach is based on the extent of the tumor. If it's above the hepatic veins, give neoadjuvant chemotherapy. And for lung metastasis, management is now response-based to avoid pulmonary radiation. So, that was our first topic in 10. What do you think? Should we continue doing these podcasts? What topics do you want us to cover? Let us know by messaging us on the Stay Current and Pediatric Surgery app or give us a shout-out on Facebook or Twitter. This chapter is created and edited by Todd Ponsky, Alex Kassar, Alex Skibbins, and myself, Ray Hinke. Remember, knowledge should be free. I was a very good to be a very good you, the one. I was a very good to be a very good to be a very good and the one. I was a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good and a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good to be a very good the one. to be a very good bar the one, the one, bar, you

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