Neuroblastoma medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Zahir Ali Shaikh, MD[2]Haytham Allaham, M.D. [3]

Overview

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, differentiation therapy, immunotherapy, and isotretinoin.[1]
  • 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
 
 
  • A combination of chemotherapy, surgery, stem cell transplantation, radiation therapy, differentiation therapy, immunotherapy, and isotretinoin
 
 
 


Management of Low Risk Neuroblastoma Patients[1]

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 among this age group demonstrate spontaneous regression.

Radiotherapy

  • Radiotherapy is generally not recommended for the management of low risk neuroblastoma patients.

Chemotherapy

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

Management of Intermediate Risk Neuroblastoma Patients[1]

Observation

  • Observation is generally not recommended for the management of intermediate risk neuroblastoma patients.

Radiotherapy

  • Indications for radiotherapy for the management of intermediate risk neuroblastoma patients include:
  • Symptomatic life-threatening neuroblastoma refractory to chemotherapy and/or surgery
  • Rapidly growing neuroblastoma associated with progressive disease symptoms

Chemotherapy

  • Chemotherapeutic agents are generally effective for the management of intermediate risk neuroblastoma patients.
  • Intermediate risk neuroblastoma patients with favorable histology are successfully managed by 4 cycles of chemotherapy following surgery.
  • Intermediate risk neuroblastoma patients with unfavorable histology are successfully managed by 8 cycles of chemotherapy following surgery.
  • Neoadjuvant chemotherapy may be used to facilitate the partial resection of previously unresectable neuroblastomas among intermediate risk patients.
  • Chemotherapeutic regimens recommended for the management of intermediate risk neuroblastoma patients may include agents such as:

Management of High Risk Neuroblastoma Patients[1]

Observation

  • Observation is generally not recommended for the management of high risk neuroblastoma patients.

Radiotherapy

  • Radiation therapy to consolidate local control after surgical resection is recommended for the management of high risk neuroblastoma patients.

Chemotherapy

  • Chemotherapy for high risk neuroblastoma patients is divided into the following three phases:
  • Induction therapy:
  • Chemotherapeutic regimens used in the induction therapy may include:
  • Consolidation therapy:
  • Consolidation therapy of high risk neuroblastoma patients consists of high dose chemotherapeutic agents administered in tandem with hematopoietic stem cell transplantation.
  • Chemotherapeutic regimens used in the consolidation therapy may include:
  • Maintenance therapy:

References

  1. 1.0 1.1 1.2 1.3 Neuroblastoma treatment–for health professionals. National Cancer Institute (2015) http://www.cancer.gov/types/neuroblastoma/hp/neuroblastoma-treatment-pdq#section/_1 Accessed on October, 8 2015


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