StayCurrentMD · Wilms Tumor
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Wilms Tumor

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What the experts said36 expert statements
Wilms tumor is the second most common intra abdominal tumor in children and fifth most common tumor in children overall
EpidemiologicalAndrew Davidoff
Approximately 75% of Wilms tumor cases occur in children younger than five years of age with a peak incidence at two to three years of age
EpidemiologicalAndrew Davidoff
Survival for patients with Wilms tumor when considered as a whole is currently greater than 90%
EpidemiologicalAndrew Davidoff
Anaplastic histology comprises only about 10% of Wilms tumor cases but contributes to over 50% of Wilms tumor mortality
EpidemiologicalAndrew Davidoff
Children with Wilms tumor typically present with an asymptomatic abdominal mass
ClinicalAndrew Davidoff
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
ClinicalAndrew Davidoff
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
GuidelineAndrew Davidoff
Intravascular tumor extension occurs in about 6% of Wilms tumor cases
EpidemiologicalAndrew Davidoff
The most common site of metastatic spread of Wilms tumor is the lungs
ClinicalAndrew Davidoff
Stage one Wilms tumors are localized tumors confined within the renal capsule
GuidelineAndrew Davidoff
Stage two Wilms tumors penetrate the renal capsule but are resected with negative margins
GuidelineAndrew Davidoff
Stage three criteria include biopsy or rupture (pre-operative or intraoperative), positive resection margin or gross residual disease, lymph node involvement, or administration of preoperative chemotherapy
GuidelineAndrew Davidoff
Metastatic disease occurs in about 12% of Wilms tumor patients and is considered stage four
EpidemiologicalAndrew Davidoff
Patients with synchronous bilateral Wilms tumor are stage five
GuidelineAndrew Davidoff
For unilateral tumors, up-front resection with regional lymph node sampling is currently the recommendation from the Children's Oncology Group
GuidelineAndrew Davidoff
Most Wilms tumors are resectable at presentation because even large tumors rarely invade surrounding structures
ClinicalAndrew Davidoff
Failure to perform up-front resection and instead administering neoadjuvant chemotherapy results in classification as stage three, mandating flank radiation and doxorubicin
GuidelineAndrew Davidoff
Treatment of favorable histology stage one or two Wilms tumor is limited to vincristine and actinomycin D
GuidelineAndrew Davidoff
For stage one tumors weighing less than 550 grams (tumor plus kidney) in patients less than 2 years of age, no adjuvant chemotherapy is given
GuidelineAndrew Davidoff
Lymph node involvement is associated with increased incidence of tumor relapse and poorer prognosis
ClinicalAndrew Davidoff
Lymph node sampling should be performed even in the absence of abnormal nodes on pre-operative imaging or gross inspection because these circumstances don't reliably predict lymph node negativity
GuidelineAndrew Davidoff
Partial nephrectomy for patients with unilateral non-syndromic disease or laparoscopic nephrectomy are not currently standard of care and should generally only be performed in the context of a clinical trial
GuidelineAndrew Davidoff
About 5% of children with Wilms tumor present with synchronous bilateral disease or stage five disease
EpidemiologicalAndrew Davidoff
Patients with bilateral Wilms tumor receive neoadjuvant chemotherapy with three drugs to shrink tumors and facilitate preservation of normal renal parenchyma
GuidelineAndrew Davidoff
A biopsy is not required in children with bilateral solid renal masses as bilateral Wilms tumor is the very likely diagnosis
GuidelineAndrew Davidoff
Biopsies of bilateral renal masses rarely detect anaplasia even when it exists in the tumor mass
ClinicalAndrew Davidoff
A biopsy if performed in bilateral disease doesn't mandate subsequent radiation as it does in patients with unilateral Wilms tumor
GuidelineAndrew Davidoff
Bilateral nephron sparing surgery should be considered in all patients with bilateral Wilms tumor after either six or 12 weeks of neoadjuvant chemotherapy
GuidelineAndrew Davidoff
Longer courses of pre-operative chemotherapy than 12 weeks are definitely discouraged in bilateral Wilms tumor
GuidelineAndrew Davidoff
Tumor extension into the renal vein and proximal inferior vena cava can in most cases be removed en bloc with the kidney and tumor
ClinicalAndrew Davidoff
Primary resection of tumors with extension above the level of the hepatic veins or into the atrium is associated with higher operative morbidity
ClinicalAndrew Davidoff
Neoadjuvant chemotherapy is generally used for intravascular tumor extension above the hepatic veins or into the atrium
GuidelineAndrew Davidoff
Thrombus extending above the hepatic veins that persists after neoadjuvant chemotherapy probably requires cardiopulmonary bypass to safely remove
GuidelineAndrew Davidoff
About 12% of Wilms tumor patients have evidence of hematogenous metastasis at diagnosis with 80% being pulmonary metastasis
EpidemiologicalAndrew Davidoff
Stage four patients with radiographic disappearance of lung metastasis or tissue confirmation of no viable tumor at week six are considered rapid responders and continue three drug chemotherapy
GuidelineAndrew Davidoff
Slow or incomplete responders to initial therapy are switched to more intensive chemotherapy and receive whole lung radiation
GuidelineAndrew Davidoff