weekly_question 13/12/2020

[Admin]

A two-year old boy presents with a 10 cm renal tumor suspected to be a Wilms tumor. You elect to perform surgical resection at diagnosis.

The operation for Wilms tumor should also include

A sampling of perirenal, periaortic and pericaval lymph nodes.

B exploration of the contralateral kidney.

C aspiration of peritoneal fluid for cytology.

D bivalving of the ipisilateral adrenal gland.

E omentectomy.

[Admin]

correct answer
A sampling of perirenal, periaortic and pericaval lymph nodes.

A review of the surgical management of Wilms tumors (WT) on Children’s Oncology Group (COG) studies shows the most common surgical protocol violation is a failure to appropriately sample lymph nodes at the initial surgery. Radical nephrectomy is the standard procedure for most unilateral WT. During nephrectomy, careful technique must be observed to ensure en bloc resection of the tumor with minimal risk of rupture. Risk factors for local tumor recurrence include tumor spillage, unfavorable histology, incomplete removal of tumor and absence of lymph node sampling. Surgical exploration should follow guidelines advocated by surgical study groups, with COG guiding practices in North America.

Although routine exploration of the contralateral kidney was originally recommended, this is not currently advocated as long as preoperative computerized tomography imaging does not suggest bilateral disease.

Formal lymph node dissection is not currently recommended although lymph node sampling from the ipsilateral renal hilum, pericaval and periaortic areas should be done for staging. The absence of lymph nodes submitted for specimen mandate treatment as Stage III disease.

In cases with tumor extension into the renal vein or retrohepatic vena cava, the thrombus should be removed en bloc with the kidney. Patients with tumor extension into either the intra- or suprahepatic vena cava should be biopsied initially and managed with chemotherapy to facilitate shrinkage of the intravascular thrombus prior to resection.

The adrenal gland should be removed if required to obtain gross total tumor resection; however, its removal is not mandatory for all tumors. Bivalving of an adjacent gland used to be recommended in ovarian tumors.

In the presence of invasion of other adjacent organs, resection of a small portion of diaphragm, psoas muscle, liver or pancreas is acceptable. However, if resection of multiple organs or extended liver resection is required to allow for complete tumor removal, primary resection is not indicated. Biopsy followed by chemotherapy may allow for a safer delayed resection.

Preoperative chemotherapy prior to resection, as advocated in Europe under SIOP, is gaining interest in North America, particularly for large bulky tumors. Children treated in Europe/SIOP with preoperative chemotherapy generally do not get a biopsy at diagnosis with a very small, but real, risk that the tumor is not a Wilms.

[Abusnaina mohammed]

A sampling of perirenal, periaortic and pericaval lymph nodes.

[Sharon]

A

[Manal Dhaiban]

A
sampling of the lymph nodes is mandatory as it is part of the staging

But in reality , it depend on the case it self,
For eg if there is any fluid in the peritoneum or if there is any sign of tumor rupture you may need to take sample

also if it is invasive and there is omental adherence to the tumor you will excised in total

if the tumor involve the upper pole , you need to remove the ipsilateral Supra renal gland

[Secundino López Ibarra]

A

[Arwa]

A : as described you need to sample 7-10 LN’s The contra lateral kidney may be assessed by radiology.

[Admin]

correct answer
A sampling of perirenal, periaortic and pericaval lymph nodes.

A review of the surgical management of Wilms tumors (WT) on Children’s Oncology Group (COG) studies shows the most common surgical protocol violation is a failure to appropriately sample lymph nodes at the initial surgery. Radical nephrectomy is the standard procedure for most unilateral WT. During nephrectomy, careful technique must be observed to ensure en bloc resection of the tumor with minimal risk of rupture. Risk factors for local tumor recurrence include tumor spillage, unfavorable histology, incomplete removal of tumor and absence of lymph node sampling. Surgical exploration should follow guidelines advocated by surgical study groups, with COG guiding practices in North America.

Although routine exploration of the contralateral kidney was originally recommended, this is not currently advocated as long as preoperative computerized tomography imaging does not suggest bilateral disease.

Formal lymph node dissection is not currently recommended although lymph node sampling from the ipsilateral renal hilum, pericaval and periaortic areas should be done for staging. The absence of lymph nodes submitted for specimen mandate treatment as Stage III disease.

In cases with tumor extension into the renal vein or retrohepatic vena cava, the thrombus should be removed en bloc with the kidney. Patients with tumor extension into either the intra- or suprahepatic vena cava should be biopsied initially and managed with chemotherapy to facilitate shrinkage of the intravascular thrombus prior to resection.

The adrenal gland should be removed if required to obtain gross total tumor resection; however, its removal is not mandatory for all tumors. Bivalving of an adjacent gland used to be recommended in ovarian tumors.

In the presence of invasion of other adjacent organs, resection of a small portion of diaphragm, psoas muscle, liver or pancreas is acceptable. However, if resection of multiple organs or extended liver resection is required to allow for complete tumor removal, primary resection is not indicated. Biopsy followed by chemotherapy may allow for a safer delayed resection.

Preoperative chemotherapy prior to resection, as advocated in Europe under SIOP, is gaining interest in North America, particularly for large bulky tumors. Children treated in Europe/SIOP with preoperative chemotherapy generally do not get a biopsy at diagnosis with a very small, but real, risk that the tumor is not a Wilms.