Editing Pediatrics: Renal and Adrenal Oncology (section)

=== Angiomyolipoma (AML) ===
* '''The renal lesions of the TSC include:'''
*# '''AML'''
*# '''Simple cysts'''
*# '''Polycystic kidney disease'''
*# '''RCC'''
** '''AML develops in up to 80% of patients with TSC.''' '''Renal cysts occur in up to 30% of patients with TSC.'''
** Mutations of 1 of 2 genes on chromosome 9 (TSC1) and chromosome 16 (TSC2) are found in 85% of TSC patients
* '''Management'''
** '''Children with growing lesions can be managed with embolization or partial nephrectomy before they become symptomatic with bleeding.'''
*** '''The risk of serious bleeding appears to correlate with a diameter > 4 cm'''
** '''mTOR inhibitors have shown promise as a new type of therapy for reducing the size of TSC-associated AMLs'''