Editing Pediatrics: Renal and Adrenal Oncology (section)

=== Management ===
* Primary treatment modalities:
*# Surgery
*# Chemotherapy
*# Radiation therapy
** The role of each depends on tumor stage, age, and biologic prognostic factors.
* '''<span style="color:#ff0000">Infants < 6 months with localized small adrenal masses can be managed with serial observation.'''
** '''Frequently associated with spontaneous regression'''
** '''Surgical resection can be avoided in 80% of such patients'''
* '''<span style="color:#ff0000">Low-Risk Disease (Stages 1 and 2): surgery (adrenalectomy)''' 
** Disease-free survival with surgical excision (adrenalectomy) alone in children with stage 1 neuroblastoma: > 90%
** '''<span style="color:#ff0000">Radical resection resulting in removal of normal organs, particularly the kidney, is not justified'''
***'''In stage 3 disease, or in stage 2 with extensive tumor around the kidney and renal vessels, preoperative treatment with chemotherapy significantly decreases the risk of nephrectomy as a result of resection of the tumor.'''
** Radiation of the local tumor bed has been advocated for treatment of residual disease in stage 2
* '''<span style="color:#ff0000">Intermediate- and High-Risk Disease (Stages 3 and 4): neoadjuvant chemotherapy followed by surgery'''
** '''Usually the safest approach for advanced tumors is to defer resection until after initial chemotherapy'''
** Neoadjuvant chemotherapy, given the efficacy of modern agents, is very successful in reducing the size of primary tumors
** There is debate regarding the extent of surgical resection that is required for stage 3 lesions
** Conflicting evidence regarding the benefit of extensive resection in children with stage 4 disease  
** '''<span style="color:#ff0000">Surgery usually is performed 13-18 weeks after initiation of chemotherapy, allowing 3-4 courses of treatment'''
** Infants age <1 year with extensive local disease or stage 4 disease comprise a special subset of patients. They have historically fared much better than children older than 1 year of age with comparable disease, but not as well as infants with stage 4S disease. It is now recognized that the biologic markers can be used to identify which infants have high-risk disease and require intensive therapy and which have intermediate-risk disease requiring less intensive therapy.
* '''Stage 4S'''
** The vast majority of these infants with '''stage 4S disease''' have tumors with entirely favorable markers, explaining their favorable behavior. However, a small fraction have adverse markers, and it is these children who have progressive disease that often is fatal.
** '''Resection of the primary is not mandatory'''
*'''Patients with extensive metastatic disease and MYCN amplification represent a high-risk group. These patients should be considered for a more aggressive treatment with multimodal therapy''', according to the risk group classification.
**Those with favorable biologic markers and no symptoms can be followed with supportive care and limited chemotherapy. 
**Intensive chemotherapy is reserved for those with adverse markers, although these infants do poorly even with therapy.
* '''Indications for adjuvant chemotherapy'''
** '''MYCN amplification and unfavorable histology'''
** '''Disease recurrence'''
*Chemotherapy
** A variety of multiagent regimens have been developed to treat high-risk patients with neuroblastoma
* '''Radiotherapy'''
** '''Radiotherapy is effective for local control in neuroblastoma,''' and risk of local relapse can be correlated with the biologic markers. '''Although irradiation has not provided a benefit in low-stage tumors, it has increased local control in children with advanced stage 4 or bulky stage 3 tumors'''