In general terms intermediate risk category NBL is treated with moderately aggressive chemotherapy (eg carboplatin, doxorubicin, etoposide and cyclophosphamide), second look surgery and attempted resection. RT for is recommended for patients older than 1 year with positive lymph nodes and is associated with improved survival.
The ANBL0531 is looking to further risk stratify within intermediate risk NBL patients and the treatment outlined below is not standard therapy in every cancer center.
Overview Intermediate risk category NBL: As specified in the current experimental COG study.
| Intermediate risk category and therapy: | ||
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Grouping |
Criteria used for grouping | Therapy |
| Group 3 | INSS Stage 3 Less than 1 year old Non-MYCN Either DI= 1 and/or UH
If there is 1p loss of heterozygosity (LOH) or unbalanced (unb)-11q LOH, patient upgraded to next risk category
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| Group 3 | INSS Stage 4 Less than I year old Non-MYCN amplified FH DI > 1a
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| Group 3 | INSS Stage 4S Infant (less than I year old) Non- MYCN amplified Either UH and any DI or FH and DI= 1
If there is 1p loss of heterozygosity (LOH) or unbalanced (unb)-11q LOH, patient upgraded to next risk category
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| Group 4 | INSS Stage 4S Symptomatic Infant (less than I year old) Unknown biologic features |
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| Group 4 | INSS Stage 4 Less than I year old Non-MYCN amplified Either DI =1 and/or UH
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| Group 4 | INSS Stage 3 1 – 1.5 years old Non-MYCN amplified UH Any ploidy
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| Group 4 | INSS Stage 4 1 – 1.5 years old Non-MYCN amplified FH DI >1
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Survival is greater than 95% for those with favorable characteristics (even if older than 1 year of age).
A3961: Infants and children with intermediate-risk neuroblastoma who received a less-intensive chemotherapy regimen had three-year overall survival rates as good as those patients in an earlier trial who received treatment that was more intensive and more toxic.
COG-ANBL0531: Phase III Study of Response and Biology-Based Combination Chemotherapy and Surgery With or Without Isotretinoin in Young Patients With Intermediate-Risk Neuroblastoma.
This COG trial is a response and biology-based trial for intermediate-risk neuroblastoma. The COG is studying a reduction of chemotherapy duration for most intermediate risk group patients, based in part on a reduction of the degree of tumor response required to stop chemotherapy (patients with 1p or 11q abnormalities will be upgraded to high-risk).
RT is reserved for patients with symptomatic life-threatening or organ-threatening tumor that does not respond rapidly to chemotherapy and/or surgery.
Aims:
- Stratify patients who have early response versus those who do not
- Attempt to give less chemotherapy and reduce risk of late effects
External Link:
Treatment of Intermediate Risk Neuroblastoma at the National Cancer Institute
