How is pediatric neuroblastoma treated?

Updated: Oct 09, 2017
  • Author: Norman J Lacayo, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Answer

Care of children with neuroblastoma is provided by a multidisciplinary team involving pediatric oncology, radiation oncologists, surgeons, and anesthesiologists, as well as nurse practitioners, nurses, pharmacists, psychologists, and physical and occupational therapists dedicated to the special needs of these children.

The table below outlines criteria for risk assignment based on the International Neuroblastoma Staging System (INSS), age, and biologic risk factors. This, in turn, determines the intensity of the therapy. These treatment strategies have been developed from more than 2 decades of experience with clinical trials in Children's Cancer Group (CCG) and Pediatric Oncology Group (POG), now known as the Children's Oncology Group (COG). Correlative biologic studies were pivotal in identifying biologic risk factors important for outcome. Currently, efforts are ongoing to develop an International Neuroblastoma Risk Group (INRG).

In addition, recently published results on correlative biologic studies and clinical outcome have lead to changes in an age cut-off of more than 365 days (365-547 d) for some patients with tumors in stages 3 and 4. [17, 18] These criteria are based on the analysis of several thousands of patients treated in cooperative group protocols in Australia, Canada, Europe, Japan, and the United States. [19]

Table 1. Current COG Neuroblastoma Risk Stratification (Open Table in a new window)

Risk Group

Stage

Age

MYCN Amplification Status

Ploidy

Shimada

Low

1

Any

Any

Any

Any

Low

2a/2b

Any

Non-amp

Any

Any

High

2a/2b

Any

Amp

Any

Any

Intermediate

3

< 547d

Non-amp

Any

Any

Intermediate

3

≥547d

Non-amp

Any

Favorable

High

3

Any

Amp

Any

Any

High

3

≥547d

Non-amp

Any

Unfavorable

High

4

< 365d

Amp

Any

Any

Intermediate

4

< 365d

Non-amp

Any

Any

High

4

365-547d

Amp

Any

Any

High

4

365-547d

Any

Diploid

Any

High

4

365-547

Any

Any

Unfavorable

Intermediate

4

365-547d

Non-amp

Hyper

Favorable

High

4

≥547d

Any

Any

Any

Low

4s

< 365d

Non-amp

Hyper

Favorable

Intermediate

4s

< 365d

Non-amp

Diploid

Any

Intermediate

4s

< 365d

Non-amp

Any

Unfavorable

High

4s

< 365d

Amp

Any

Any


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