Neuroblastoma medical therapy
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Risk calculators and risk factors for Neuroblastoma medical therapy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
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, differentiation therapy, immunotherapy, and isotretinoin.[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, 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 | |||||||||||||||||||||||||||
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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 may include agents such as:
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
- Rapidly growing neuroblastoma associated with progressive disease symptoms
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:
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:
- 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.