Simplified overview of low risk category NBL and therapy:
NB Even though ANBL0531 had "intermediate risk" in its title, this classification of low risk NBL was developed for this study which is now closed to accrual.
| Low risk category and therapy: | ||
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Grouping |
Criteria used for grouping | Therapy |
| Group 1 | INSS Stage 1 Any MYCN status Any age
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| Group 1 | INSS Stage 2A or 2B > or = 50% resected Non-MYCN amplified Any histology Any DNA Index (DI) Any age
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| Group 1 | INSS Stage 4S Infant (less than I year old) Non-MYCN amplified FH DI >1
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| Group 2 | INSS Stage 2A or 2B Less than 50% resected or biopsy only Non-MYCN amplified 1 - 12 years old Any histology Any DI |
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| Group 2 | INSS Stage 3 Less than I year old Non-MYCN amplified FH DI >1
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| Group 2 | INSS Stage 3 I - 12 years old Non-MYCN amplified FH
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| Group 2 | INSS Stage 4S Symptomatic Infant (less than I year old) Non-MYCN amplified FH DI >1 |
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Treatment options for low risk NBL may include:
Observation without biopsy:
- Appropriate for infants younger than 6 months with disease limited to the adrenal gland less than 3cm in diameter.
Surgery:
- In localized disease, radical surgical excision is the management of choice.
- Complete or even partial resection alone is usually sufficient therapy.
- Curative resection is usually possible for Stage 1 disease and sometimes for Stage 2A.
- Surgery alone can be used to treat biologically favorable and localized tumor which is incompletely resected (stage 2A and stage 2B).
Chemotherapy:
- In infants with unresectable disease, chemotherapy can downstage the tumor and make excision possible.
- Used for symptomatic disease or unresectable, progressive disease after surgery
- Overall survival rate greater than 95%.
- One course of chemotherapy consists of four 3 week cycles.
- Each cycle has different combination of 4 drugs:
COG clinical trials:
COG-ANBL0531 (therapeutic trial) and COG-ANBL00B1 (biology study required for entry onto COG-ANBL0531):
A risk group classification system was developed for this COG study (see table above). Patients classified as low risk were in treatment Group 1 or Group 2.
Group 1 was treated with surgery followed by observation. Chemotherapy was recommended only for life-threatening or organ-threatening symptoms that could not be relieved by safe surgical resection of the mass. Life-threatening or organ-threatening symptoms included respiratory distress, renal or bowel ischemia, spinal cord compression, gastrointestinal or genitourinary obstruction, and coagulopathy.
Group 2 patients were treated with surgery followed by two cycles of chemotherapy, and if needed additional cycles of chemotherapy until partial response was obtained. If the tumor had 1p or 11q loss of heterozygosity (LOH) or the LOH studies were not performed, the patient was placed in the treatment Group 3 (intermediate risk category) and received four cycles of chemotherapy.
Chemotherapy was given for two cycles (6 weeks) and consisted of moderate doses of carboplatin, cyclophosphamide, doxorubicin, and etoposide. The cumulative dose of each agent was kept low to reduce the risk of late effects, as used in prior COG trials (COG-P9641 and COG-A3961).
RT was reserved for patients with symptomatic life-threatening or organ-threatening tumor that does not respond rapidly enough to chemotherapy and/or surgery.
External Link:
Treatment of low risk neuroblastoma at the National Cancer Institute
