Wilms Tumor
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Question: 1/12
A three-year old child presents with a large renal mass suspected to be a Wilms tumor. Surgical resection is performed and pathologic evaluation shows completely resected Wilms tumor with focal penetration of the renal capsule. Perirenal, periaortic and pericaval lymph nodes are all negative. There are no histologic foci of anaplasia.

According to the Childrens Oncology Group, the capsular penetration seen on histologic exam results in which stage and what treatment?
a
stage I and no chemotherapy
b
stage II and postoperative chemotherapy is VCR/AMD
c
stage II and postoperative chemotherapy is VCR/AMD plus flank radiation
d
stage III and postoperative chemotherapy is VCR/AMD/DOX
e
stage III and postoperative VCR/AMD/DOX plus flank radiation
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Question: 2/12

Children with which of the following conditions should receive screening renal ultrasounds for nephroblastoma (Wilms tumor)?
a
a teenager who had unilateral Wilms’ at four years of age
b
Denys-Drash syndrome
c
end stage renal failure
d
cryptorchidism
e
isolated Wilms’ tumor in a sibling
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Question: 3/12
An 18-month old male is diagnosed with a right renal mass. You successfully perform a right radical nephrectomy with lymph node sampling. The pathology returns stage I favorable histology Wilms tumor with a weight of 320 g and neither loss of heterozygosity (LOH) nor loss of imprinting (LOI) of 11p15.

According to Fernandez (Ann Surg 265:835, 2017), which adjuvant therapy will result in the highest overall survival with the lowest toxicity?
a
vincristine and dactinomycin for 18 weeks
b
observation without adjuvant therapy
c
vincristine, dactinomycin and doxorubicin for 24 weeks
d
vincristine, dactinomycin for 18 weeks and radiation to the right flank
e
radiotherapy to the right flank only
SUBMIT ANSWER
Question: 4/12
A four-year old child with favorable histology nephroblastoma experiences tumor spillage at the time of surgical nephrectomy.

Current Children’s Oncology Group recommendations for radiation therapy in Wilms tumor patients specify that
a
no radiation therapy is necessary as long as the spill was confined to the flank.
b
no radiation therapy is necessary because the patient will now be upstaged and receive doxorubicin.
c
postoperative radiation therapy should be given as soon as possible after surgery.
d
spillage does not increase the risk of local relapse.
e
spillage does not affect outcome.
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Question: 5/12
A 10-month old male with aniridia is found to have a right sided renal mass on screening ultrasound. Magnetic resonance imaging shows bilateral renal masses.
MRI

The most appropriate management of this patient with bilateral renal masses is
a
unilateral needle biopsy.
b
bilateral needle biopsies.
c
open unilateral biopsy
d
open biopsy with lymph node sampling.
e
chemotherapy.
SUBMIT ANSWER
Question: 6/12
A three-year old male undergoes left nephrectomy for a favorable histology Wilms tumor.

The genetic characteristic which portends poorer prognosis in Wilms tumor is
a
loss of heterozygosity 1 p and 16 q.
b
increased WT-1 expression.
c
N-myc amplification.
d
decreased WT-2 expression.
e
presence of RET oncogene.
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Question: 7/12
Following nephrectomy, a four-year old male with favorable histology Wilms tumor has a normal chest plain radiograph but multiple lung metastases visible on chest computerized tomography.

The most effective treatment to improve event free survival in this patient with metastatic Wilms tumor is
a
surgical resection of nodules.
b
whole lung radiation.
c
vincristine, dactinomycin and doxorubicin.
d
vincristine, dactinomycin and whole lung radiation.
e
proton beam irradiation.
SUBMIT ANSWER
Question: 8/12

The guidelines from the Children’s Oncology Group call for preoperative chemotherapy when
a
the primary tumor extends across the midline.
b
the patient is over five years of age.
c
there is a history of Wilms’ tumor in the family.
d
there are pulmonary metastases at presentation.
e
tumor thrombus extends into the right atrium.
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Question: 9/12
A four-year old child presents with a 14 cm mass involving the hilum of the right kidney with radiographic features consistent with Wilms tumor. The chest computerized tomography (CT) scan at diagnosis shows a single 1.2 cm pulmonary nodule in the left lower lobe felt to be consistent with metastatic disease. The child undergoes a right radical nephrectomy with lymph node dissection. Pathology shows favorable histology with no loss of heterozygosity at chromosomes 1p and 16q. Two of five lymph nodes are positive for tumor.
chest CT
After completing adjuvant chemotherapy with vincristine, dactinomycin and doxorubicin, a chest CT shows persistent residual at the site of the left lower lobe pulmonary lesion.

Your recommendation for management of this patient with metastatic Wilms tumor is
a
lung radiation focused on the left lower lobe.
b
whole lung radiation.
c
thoracoscopic biopsy of the residual lesion.
d
thoracotomy with lung palpation and resection of all suspicious nodules.
e
bilateral thoracotomy or median sternotomy with whole lung palpation and resection of all suspicious nodules.
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Question: 10/12
A two-year old female presents with a unilateral five cm renal tumor confined to the upper pole of the left kidney. Computerized tomography reveals multiple lesions in both lungs. She is otherwise asymptomatic.

The next best step in management of this patient with a metastatic renal tumor is
a
open renal and lung biopsy.
b
renal biopsy followed by adjuvant chemotherapy.
c
left total nephroureterectomy with lymph node sampling.
d
left partial nephrectomy.
e
intraoperative biopsy of left tumor followed by nephrectomy if Wilms tumor.
SUBMIT ANSWER
Question: 11/12
A four-year old male is diagnosed with a large right sided renal tumor. Computerized tomography scan of the chest and ultrasound evaluation of the inferior vena cava reveals no metastatic lesion or tumor thrombus.

The most appropriate surgical management includes right nephrectomy and
a
left renal exploration.
b
intraoperative ultrasound of the retrohepatic vena cava.
c
right hilar and retroperitoneal lymph node sampling.
d
multiple peritoneal biopsies.
e
omentectomy.
SUBMIT ANSWER
Question: 12/12
A 10-month old boy is found to have an abdominal mass on physical exam. An abdominal computerized tomography (CT) scan is obtained.
CT scan
The other abdominal organs, including the right kidney and inferior vena cava appear normal. A chest CT scan was also normal.

The recommended next step for this patient with a presumed cystic renal tumor is
a
biopsy of the tumor.
b
initial treatment with vincristine and dactinomycin.
c
left partial nephrectomy.
d
left nephrectomy and retroperitoneal lymph node sampling.
e
left nephrectomy with biopsy of right kidney.
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Wilms Tumor, question 15
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Wilms Tumor, question 3
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Wilms Tumor, question 5
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Wilms Tumor, question 8
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Wilms Tumor, question 10
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Wilms Tumor, question 13
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Wilms Tumor, question 14
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Wilms Tumor, question 15
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Wilms Tumor, question 17
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Wilms Tumor, question 18
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Wilms Tumor, question 19
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Wilms Tumor, question 20
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Pediatric Renal Tumors, question 1
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