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


==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.<ref name="gov">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</ref>
* The algorithm below summarizes the management approach for neuroblastoma patients:
<br>
{{Family tree/start}}
{{familytree  | | | | | | Y01 | | | | | | | | Y01=<div style="width: 20em; padding:0em;">'''Children's Oncology Group risk stratification'''</div>}}
{{familytree  | | |,|-|-|-|+|-|-|-|.| | | | }}
{{familytree  | | X01 | |  X02 | | X03 | | | |X01=<div style="width: 20em; padding:0em;">'''Low risk patients'''</div>| X02=<div style="width: 20em; padding:0em;">'''Intermediate risk patients'''</div>| X03=<div style="width: 20em; padding:0em;">'''High risk patients'''</div>}}
{{familytree|boxstyle= border-top: 0px;| | A01 | |  A02 | | A03 | | | |A01=<div style="width: 20em; padding:0em;text-align:left">
*'''Surgery followed by chemotherapy'''
*'''Chemotherapy with or without surgery'''
*'''Observation without biopsy'''
</div>|A02=<div style="width: 20em; padding:0em;text-align:left">
*'''Chemotherapy with or without surgery'''
*'''Surgery and observation'''
*'''Radiation therapy'''
</div>|A03=<div style="width: 20em; padding:0em;text-align:left">
*'''A combination of chemotherapy, surgery, stem cell transplantation, radiation therapy, differentiation therapy, immunotherapy, and isotretinoin'''
</div>}}
{{Family tree/end}}
<br>


Low risk neuroblastoma patients are managed with either observation or surgical resection of the tumor.
===Management of Low Risk Neuroblastoma Patients<ref name="gov">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</ref>===
Intermediate risk neuroblastoma patients are managed with neoadjuvant chemotherapy in advance of a definitive surgical resection.
====Observation====
High risk neuroblastoma patients are managed with a combination of surgery, chemotherapy, radiation therapy, hematopoietic stem cell transplantation, and immunotherapy.
* 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.
====Radiotherapy====
* Radiotherapy is generally not recommended among low risk neuroblastoma patients.
====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 regimens recommended for the management of  neuroblastoma include:
:* Carboplatin
:* Cyclophosphamide
:* Doxorubicin
:* Etoposide


Children's Oncology Group Risk Stratification
===Management of Intermediate Risk Neuroblastoma Patients<ref name="gov">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</ref>===
====Observation====
* Observation is generally not recommended among intermediate risk neuroblastoma patients.
====Radiotherapy====
* Indications for radiotherapy among intermediate risk neuroblastoma patients include:
:* Symptomatic life-threatening neuroblastoma refractory to chemotherapy and/or surgery
:* Progressive rapidly growing neuroblastoma


====Chemotherapy====
* Chemotherapeutic agents are generally effective in the treatment 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  neuroblastoma include:
:* Carboplatin
:* Cyclophosphamide
:* Doxorubicin
:* Etoposide


 
===Management of High Risk Neuroblastoma Patients<ref name="gov">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</ref>===
{{Family tree/start}}
====Observation====
{{familytree  | | | | | | Y01 | | | | | | | | Y01=<div style="width: 20em; padding:0.5em;">'''Children's oncology group risk stratification'''</div>}}
* Observation is generally not recommended among high risk neuroblastoma patients.
{{familytree  | | |,|-|-|-|+|-|-|-|.| | | | }}
====Radiotherapy====
{{familytree  | | X01 | |  X02 | | X03 | | | |X01=<div style="width: 20em; padding:0.5em;">'''Low risk patients'''</div>| X02=<div style="width: 20em; padding:0.5em;">'''Intermediate risk patients'''</div>| X03=<div style="width: 20em; padding:0.5em;">'''High risk patients'''</div>}}
* Radiation therapy to consolidate local control after surgical resection is recommended for the management of high risk neuroblastoma patients.
{{familytree|boxstyle= border-top: 0px;| | A01 | |  A02 | | A03 | | | |A01=<div style="width: 20em; padding:0.5em;">
====Chemotherapy====
'''Craniospinal radiation <br>{{or}}<br> Chemoradiotherapy followed by additional [[#Chemotherapy|chemotherapy]]'''</div>|A02=<div style="width: 15em; padding:0.5em;">'''[[#Radiation Therapy|Craniospinal radiation]] followed by chemotherapy'''</div>|A03=<div style="width: 15em; padding:0.5em;">'''[[#Radiation Therapy|Craniospinal radiation]] followed by chemotherapy'''</div>}}
* Chemotherapy for high risk neuroblastoma patients is divided into the following three phases:
{{Family tree/end}}
:* '''Induction therapy:'''
::* Chemotherapeutic regimens used in the induction therapy may include:
:::* Cisplatin
:::* Etoposide
:::* Vincristine
:::* Cyclophosphamide
:::* Doxorubicin
:::* Topotecan
:* '''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:
:::* Carboplatin
:::* Etoposide
:::* Melphalan
:::* Busulfan
:::* Vincristine
:::* Irinotecan
:* '''Maintenance therapy:'''
::* A combination of differentiation therapy (GM-CSF and IL-2), isotretinoin, and immunotherapy (chimeric anti-GD2 antibody-ch14.18) are adminstered following hematopoietic stem cell transplantation to improve the survival of high risk neuroblastoma patients.

Latest revision as of 00:33, 10 October 2015

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.[1]

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.[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
 
  • Chemotherapy with or without surgery
  • Surgery and observation
  • Radiation therapy
 
  • 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 in this age group demonstrate spontaneous regression.

Radiotherapy

  • Radiotherapy is generally not recommended among low risk neuroblastoma patients.

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 regimens recommended for the management of neuroblastoma include:
  • Carboplatin
  • Cyclophosphamide
  • Doxorubicin
  • Etoposide

Management of Intermediate Risk Neuroblastoma Patients[1]

Observation

  • Observation is generally not recommended among intermediate risk neuroblastoma patients.

Radiotherapy

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

Chemotherapy

  • Chemotherapeutic agents are generally effective in the treatment 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 neuroblastoma include:
  • Carboplatin
  • Cyclophosphamide
  • Doxorubicin
  • Etoposide

Management of High Risk Neuroblastoma Patients[1]

Observation

  • Observation is generally not recommended among 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:
  • Cisplatin
  • Etoposide
  • Vincristine
  • Cyclophosphamide
  • Doxorubicin
  • Topotecan
  • 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:
  • Carboplatin
  • Etoposide
  • Melphalan
  • Busulfan
  • Vincristine
  • Irinotecan
  • Maintenance therapy:
  • A combination of differentiation therapy (GM-CSF and IL-2), isotretinoin, and immunotherapy (chimeric anti-GD2 antibody-ch14.18) are adminstered following hematopoietic stem cell transplantation to improve the survival of high risk neuroblastoma patients.
  1. ↑ 1.0 1.1 1.2 1.3 1.4 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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