weekly_question 8/5/2022

[Admin]

A 10-month old boy is found to have an abdominal mass on physical exam. An abdominal computerized tomography (CT) scan is obtained. 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 left renal tumor is



A biopsy of the tumor.

B left partial nephrectomy.

C initial treatment with vincristine and dactinomycin.

D left nephrectomy with biopsy of right kidney.

E left nephrectomy and retroperitoneal lymph node sampling.

[Admin]

correct answer
E left nephrectomy and retroperitoneal lymph node sampling.

Cystic renal tumors can be a diagnostic and therapeutic challenge with a spectrum ranging from

cystic nephroma (CN) - benign cystic tumors with only mature elements in the septae and cystic tissue
cystic partially differentiated nephroblastoma (CPDN) - intermediate risk tumors with immature components in the septae and cystic tissue but no solid components
cystic Wilms tumor (CWT) - malignant lesions with cystic and solid components
Although CN tends to occur in children under one year of age and a solid component may be an indicator of CWT, radiographically the three types may be indistinguishable.

In the United States, left nephrectomy would be the most appropriate initial therapy. Preoperative chemotherapy would be a consideration by SIOP standards if CWT was suspected based upon solid components. However, a decision could be made to forego this if the imaging did not have solid components and CN or CPDN were suspected. Biopsy is inappropriate and would upstage the patient if the tumor were subsequently determined to be CWT. The decision for postoperative chemotherapy would be based on appropriate staging for WT if that were the pathology. No chemotherapy would be needed for CN.

Although controversial, the literature appears to indicate that chemotherapy may not be needed for CPDN after resection. Blakely published a report from the Wilms Tumor Study Group in 2003 reviewing 21 patients with CPDN. Thirteen patients received chemotherapy despite ten of the tumors being Stage I. Four patients experienced toxicity. Eight patients received no chemotherapy. All survived with no recurrences. In a retrospective review from the SIOP/GPOH Study group in 2007, 14 patients were identified with CN or CPDN. Two patients had received chemotherapy before operation and one after surgery. Two patients underwent partial nephrectomy. Survival was 100% and there was no progression of disease.

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C

[Ismailmohamed]

C answer may be according to SIOP protocol initially chemotherapy as CT show large wilms tumor but I will choose Left nephrectomy with lymph node sample as there is no invasion of great vessels or chest metastasis.

[Radwan suleiman abukarsh]

E

[Manal Dhaiban]

C As per SIOP
willms diagnosed by radiology no need to biopsy between 6 months and up to 7 years
start with chemo then re asses in 4 cycle then plan
radical nephroureterectomy with LN Sampling

[Admin]

correct answer
E left nephrectomy and retroperitoneal lymph node sampling.

Cystic renal tumors can be a diagnostic and therapeutic challenge with a spectrum ranging from

cystic nephroma (CN) - benign cystic tumors with only mature elements in the septae and cystic tissue
cystic partially differentiated nephroblastoma (CPDN) - intermediate risk tumors with immature components in the septae and cystic tissue but no solid components
cystic Wilms tumor (CWT) - malignant lesions with cystic and solid components
Although CN tends to occur in children under one year of age and a solid component may be an indicator of CWT, radiographically the three types may be indistinguishable.

In the United States, left nephrectomy would be the most appropriate initial therapy. Preoperative chemotherapy would be a consideration by SIOP standards if CWT was suspected based upon solid components. However, a decision could be made to forego this if the imaging did not have solid components and CN or CPDN were suspected. Biopsy is inappropriate and would upstage the patient if the tumor were subsequently determined to be CWT. The decision for postoperative chemotherapy would be based on appropriate staging for WT if that were the pathology. No chemotherapy would be needed for CN.

Although controversial, the literature appears to indicate that chemotherapy may not be needed for CPDN after resection. Blakely published a report from the Wilms Tumor Study Group in 2003 reviewing 21 patients with CPDN. Thirteen patients received chemotherapy despite ten of the tumors being Stage I. Four patients experienced toxicity. Eight patients received no chemotherapy. All survived with no recurrences. In a retrospective review from the SIOP/GPOH Study group in 2007, 14 patients were identified with CN or CPDN. Two patients had received chemotherapy before operation and one after surgery. Two patients underwent partial nephrectomy. Survival was 100% and there was no progression of disease.