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Overview

Children oncology group (COG) risk stratification system determines the protocol of management used for neuroblastoma patients. Low risk neuroblastoma patients are usually managed with either observation or surgical resection of the tumor. Intermidiate risk neuroblastoma patients are usually managed with neoadjuvant chemotherapy in advance of a definitive surgical resection. Whereas high risk neuroblastoma patients are usually managed with a combination of surgery, chemotherapy, radiation therapy, hematopoietic stem cell transplantation, and immunotherapy.

Medical Therapy

Risk Stratification

  • Children oncology group (COG) risk stratification system determines the protocol of management used for neuroblastoma patients.
  • Low risk neuroblastoma patients are usually managed by either observation or surgical resection of the tumor.
  • Intermediate risk neuroblastoma patients are usually managed by neoadjuvant chemotherapy in advance of a definitive surgical resection.
  • High risk neuroblastoma patients are usually managed by a combination of surgery, chemotherapy, radiation therapy, hematopoietic stem cell transplantation, and immunotherapy.
  • The algorithm below summarizes the management approach for neuroblastoma patients:


 
 
 
 
 
Children's oncology group risk stratification
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Low risk patients
 
Intermediate risk patients
 
High risk patients
 
 
 
 
  • Surgery followed by chemotherapy
  • Chemotherapy with or without surgery
  • Observation without biopsy
 
  • Chemotherapy with or without surgery
  • Surgery and observation
  • Radiation therapy
 
  • A combination of chemotherapy, surgery, stem cell transplantation, radiation therapy, immunotherapy, and isotretinoin
 
 
 


Management of Low Risk Neuroblastoma Patients

Observation

  • Low risk neuroblastoma patients younger than 6 months of age may be safely observed without obtaining a definitive histologic diagnosis or performing any surgical intervention.
  • Observation among such patients avoids potential surgical complications, as the majority of neuroblastomas occurring in this age group demonstrate spontaneous regression.

Chemotherapy

  • Indications for chemotherapy among low risk neuroblastoma patients include:
  • Stage 1 or stage 2 tumors associated with MYCN amplification
  • Age older than 18 months presenting with a stage 2B tumor and an unfavorable histology
  • Symptomatic patients due to spinal cord compression, respiratory compromise, and hepatic infiltration
  • Chemotherapeutic agents recommended for the management of neuroblastoma include:
  • Carboplatin
  • Cyclophosphamide
  • Doxorubicin
  • Etoposide

Radiotherapy

  • Radiotherapy is not recommended among low risk neuroblastoma patients.
  • Surgical intervention alone may be curative as a single therapeutic modality for the management of low risk neuroblastoma patients.


Management of Intermediate Risk Neuroblastoma Patients

Radiation therapy=

  • Indications for radiotherapy among intermediate risk neuroblastoma patients include:
  • Symptomatic life-threatening neuroblastoma refractory to chemotherapy and/or surgery
  • Progressive rapidly growing neuroblastoma