Editing Pediatrics: Renal and Adrenal Oncology (section)

=== Renal Cell Carcinoma ===
* '''RCC is the most likely diagnosis in a child age >12 with a renal mass'''
* Staging
** Same TNM system as that used for adults
** 63% of children present with locally advanced or metastatic disease (N1 or M1)
* '''Pathology'''
** '''Most common histology seen in pediatric RCC is translocation''' (47% of cases)
*** Clear cell RCC is rare in pediatrics, unlike adults in whom clear cell RCC is the most common histology,
** '''[Campbell’s] Higher incidence of papillary RCC in children'''
*** These tumors are genetically unique in that they have chromosome translocations involving a common breakpoint in the TFE gene located at Xp11.2
** '''Another type of RCC more often seen in children is renal medullary carcinoma'''
*** Median age at presentation is 13 years
*** Found in patients with sickle cell hemoglobinopathy
* Diagnosis and Evaluation
** History and Physical Exam
*** Children tend to present with an abdominal mass or hematuria
** Imaging
*** Cannot differentiate RCC from other solid renal tumors
* '''Management'''
** '''Nephrectomy is mainstay of treatment'''
*** '''NSS should only be advocated in highly select patients when the lesion can be completely excised and the surgeon has adequate experience'''
**** Important to remember that the application of MIS and NSS to children with RCC is likely limited as children are frequently treated under the assumption of a preoperative diagnosis of WT.
** '''LN sampling is recommended at the time of nephrectomy in all cases of suspected RCC''' (regardless of preoperative imaging, tumor size, NSS, MIS) '''due to the high prevalence of nodal involvement'''
*** In adults, LN involvement is rare in smaller tumors, which is not the case in children. Almost half (47.5%) of children with T1 tumors have regional LN involvement; pathologically involved LNs were missed on preoperative imaging alone in >42% of cases