Simplified overview of low risk category NBL and therapy:
NB Even though ANBL0531 has "intermediate risk" in its title, this classification of low risk NBL was developed for this study.
| 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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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.
If biologically favorable and localized tumor which is incompletely resected (stage 2A and stage 2B) - treat with surgery alone.
In infants with unresectable disease, chemotherapy can downstage the tumor and make excision possible.
Overall survival rate greater than 95%.
One course of chemotherapy consists of four 3 week cycles.
Each cycle has different combination of 4 drugs:
Current COG clinical trials:
COG-ANBL0531 (therapeutic trial) and COG-ANBL00B1 (biology study required for entry onto COG-ANBL0531):
A new risk group classification system has been developed for this COG study (see table above). Patients classified as low risk are now in treatment Group 1 or Group 2.
Group 1 is treated with surgery followed by observation. Chemotherapy is recommended only for life-threatening or organ-threatening symptoms that cannot be relieved by safe surgical resection of the mass. Life-threatening or organ-threatening symptoms include respiratory distress, renal or bowel ischemia, spinal cord compression, gastrointestinal or genitourinary obstruction, and coagulopathy.
Group 2 patients are treated with surgery followed by two cycles of chemotherapy, and if needed additional cycles of chemotherapy until partial response is obtained. If the tumor has 1p or 11q loss of heterozygosity (LOH) or the LOH studies are not performed, the patient is placed in the treatment Group 3 (intermediate risk category) and receives four cycles of chemotherapy.
Chemotherapy is given for two cycles (6 weeks) and consists of moderate doses of carboplatin, cyclophosphamide, doxorubicin, and etoposide. The cumulative dose of each agent is kept low to reduce the risk of late effects, as used in prior COG trials (COG-P9641 and COG-A3961).
RT is 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
