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Neuroblastoma

 

 

Treatment Overview

Treatment of NBL depends on the risk stratification.

May involve surgery, chemotherapy, bone marrow transplant and radiation therapy (RT).

Chemotherapy is the major modality of treatment for most patients.

CCG-3881 regimen was a study that showed improved survival with chemotherapy and used four of the most active agents in neuroblastoma:

In patients with localized unresectable disease combination chemotherapy and second look surgery has a good chance of achieving local control. 

Infants with disseminated disease may be cured by chemotherapy alone. 

For older children with disseminated disease chemotherapy can produce partial and complete remissions, but relapse is very common.

 

Simplified overview of therapy for the different neuroblastoma risk categories:

Risk Category

Therapy

Low risk

 

  • INSS Stage 1 treated with surgery alone - chemotherapy only for relapse

 

  • INSS Stage 2A treated with surgery followed by minimal chemotherapy

 

  • INSS Stage 2B or 3 in infants without N-myc amplification treated with surgery followed by minimal chemotherapy

 

  • INSS 4S - appropriate to observe and treat for symptomatic relief only.

 

  • In localized disease radical surgical excision is the management of choice.  In infants with unresectable disease chemotherapy can downstage disease and make excision possible.

 

Intermediate Risk

 

  • Moderately aggressive chemotherapy (eg doxorubicin, cyclophosphamide, cisplatin)

 

  • Second look surgery and attempted resection.

 

  • RT for patients older than 1 year with positive lymph nodes is associated with improved survival.

 

High Risk

 

Therapy consists of all of the following:

  • Aggressive multiagent chemotherapy
  • Second look surgery
  • High dose myeloablative chemotherapy followed by stem cell rescue
  • Tumor bed RT
  • Biologic therapy with antibodies and cis-retinoic acid.

 

 

Links:

 

Treatment Option Overview for Neuroblastoma at the NCI

 

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