Editing Pediatrics: Renal and Adrenal Oncology (section)

=== Management ===

==== Initial Management ====
*'''<span style="color:#ff0000">Principles of initial treatment of unilateral, non-syndromic tumors, even in patients with metastatic disease:'''
*#'''<span style="color:#ff0000">Upfront open transperitoneal radical, adrenal-sparing, nephrectomy with''' 
*#'''<span style="color:#ff0000">Lymph node sampling and''' 
*#'''<span style="color:#ff0000">Without tumour spillage''' 
*#* '''In general, Children's Oncology Group advocates for upfront nephrectomy, confirmation of diagnosis and then chemotherapy'''
*#**International Society of Pediatric Oncology (SIOP) advocates for an assumed diagnosis of WT, followed by pre-operative chemotherapy and then surgery for all patients.
*#*** Outcomes are similar, regardless of which protocol is used but there are differences in cumulative doses of therapies and the number of patients exposed to various therapies.
*#*** In North America children, and adolescents are generally treated per COG guidelines
* '''<span style="color:#ff0000">Nephron-sparing surgery'''
**'''<span style="color:#ff0000">Indications (4):'''
**# '''<span style="color:#ff0000">Bilateral tumors'''
**# '''<span style="color:#ff0000">Tumor in a solitary kidney'''
**# '''<span style="color:#ff0000">Pre-disposition syndrome'''
**# '''<span style="color:#ff0000">Clinical trials'''
*** '''Typically, patients undergoing nephron-sparing surgery will undergo preoperative chemotherapy''' with an assumed diagnosis of WT (COG does not recommend routine biopsy in this setting) '''to allow tumor shrinkage to preserve as much normal renal tissue as possible'''
* '''<span style="color:#ff0000">Approach'''
**'''<span style="color:#ff0000">Open radical nephrectomy is standard'''
***'''Extreme caution must be used applying minimally-invasive surgery to nephron-sparing surgery in children,''' and neither is advocated nor been studied by COG or SIOP
* '''<span style="color:#ff0000">Principles in Wilm's Tumor Surgery (6)'''
*#'''<span style="color:#ff0000">Thorough exploration of the abdominal cavity is necessary to exclude local tumor extension, liver and nodal metastases, and peritoneal seeding</span>'''
*#* Accurate staging is essential for the subsequent determination of the need for radiation therapy and the appropriate chemotherapy regimen.
*# '''<span style="color:#ff0000">Perform surgery without tumor spillage</span>'''
*#* Local recurrence is increased in patients with local tumor spillage, and is classified as stage III disease. 2-year survival rate after local recurrence is 43%
*#'''<span style="color:#ff0000">Palpate the renal vein and IVC</span>''' to exclude intravascular tumor extension before vessel ligation.
*#'''<span style="color:#ff0000">Lymph node sampling must be included at the same time as resection even with nephron-sparing.'''
*#* '''Selective sampling of suspicious nodes is an essential component of local tumor staging'''.
*#** '''Formal retroperitoneal lymph node dissection is not recommended'''
*#*The extent of LN dissection and location of nodal sampling need to be better defined to allow further study.
*#'''<span style="color:#ff0000">Adrenal gland can be spared</span> without increasing the risk for tumor spill or recurrence if it is not in close proximity to the tumor'''
*#'''<span style="color:#ff0000">Routine exploration of the contralateral kidney at the time of nephrectomy is not necessary when preoperative imaging with CT or MRI demonstrates a normal contralateral kidney</span>'''

==== Adjuvant Treatment[https://wjps.bmj.com/content/2/3/e000038 §] ====
* '''<span style="color:#ff0000">Chemotherapy'''
** '''<span style="color:#ff0000">Routinely used in all patients with WT except those at a very low risk'''
*** '''Very low risk criteria (4):'''
***# '''Age < 2 at diagnosis and'''
***# '''Stage I with favorable histology and'''
***# '''Tumor weighing <550 g and'''
***# '''Confirmed negative lymph nodes'''
** '''<span style="color:#ff0000">Regimen: vincristine, doxorubicin, +/- dactinomycin/adriamycin'''
*** '''Generally:'''
**** '''Stage I and II: vincristine and doxorubicin'''
**** '''Stage III+: vincristine, doxorubicin, and dactinomycin/adriamycin'''
*** '''Anaplastic/unfavourable histology: vincristine, doxorubicin, cyclophosphamide, etopiside'''
* '''Radiation: all patients with tumor stage III'''+
* Details available on UpToDate.com

==== Pre-operative Chemotherapy ====
* '''<span style="color:#ff0000">Indications</span>''' (Campbell’s and [https://wjps.bmj.com/content/2/3/e000038 §]) '''(7):'''
*# '''<span style="color:#ff0000">Solitary kidney'''
*# '''<span style="color:#ff0000">Bilateral tumours'''
*# '''<span style="color:#ff0000">Tumor extension into the IVC above the hepatic veins'''
*# '''<span style="color:#ff0000">Tumor involving contiguous structures whereby removing the kidney tumor would require removal of the other organs, such as spleen, pancreas, or colon'''
*# '''<span style="color:#ff0000">Tumors inoperable at surgical exploration'''
*# '''<span style="color:#ff0000">Tumour not amenable to partial nephrectomy [in patients with indication for nephron-sparing surgery]'''
*#* Most Wilms tumors are too large at diagnosis to allow partial nephrectomy. After preoperative chemotherapy, partial nephrectomy can be performed in 10-5% of patients
*# '''<span style="color:#ff0000">Extensive pulmonary metastases'''
** '''Bilateral tumors'''
*** Occurs in 5-10% of WT cases
*** Associated with worse outcomes
*** Increased risk of renal impairment
*** MRI is the imaging modality of choice
*** '''Because bilateral Wilms is overwhelmingly likely in a child 1-7 years old with bilateral renal masses,''' '''preoperative biopsy is not indicated''' and will up stage the disease to local stage III
*** '''Preoperative chemotherapy''' with vincristine, actinomycin and doxorubicin '''will be given''' for 2 cycles (6 weeks), after which imaging is repeated.
*** '''LN sampling on each side must be included with surgical intervention for patients with bilateral renal tumors, just like all patients with WT'''
* '''Tumors not responding to chemotherapy require bilateral open biopsy to determine histology.'''
** '''Open biopsies are recommended because they are more accurate than percutaneous needle biopsies when assessing for anaplasia, and bilateral biopsies are recommended'''