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9 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Intermediate-risk neuroblastoma sits between the tumours that go away on their own and the high-risk disease that needs everything. A few cycles of moderate chemotherapy followed by surgery cure most children, and trials have spent twenty years showing how few cycles are enough.
The intermediate-risk group takes in L2 tumours in children with unfavourable histology or 11q aberration, L2 tumours in children over 18 months, metastatic stage M disease in infants under 18 months, and stage MS with unfavourable biology, all without MYCN amplification. These tumours will not regress reliably and cannot be removed safely at diagnosis, but they respond to chemotherapy and rarely relapse after it. The question the trials have asked is not which drug but how little: each cycle of carboplatin, etoposide, cyclophosphamide and doxorubicin adds hearing loss, infertility and cardiac risk to a child who will live for seventy years.
COG A3961, reported in the New England Journal of Medicine in 2010, gave 479 children four or eight cycles of that four-drug chemotherapy according to histology and ploidy and operated when the tumour became resectable: three-year overall survival was 96 percent and event-free survival 88 percent, establishing a biology-based reduction of therapy. ANBL0531 then cut further, giving two cycles to the most favourable subset and adding response-based escalation for the rest, with three-year event-free survival 83.2 percent and overall survival 94.9 percent; infants with stage M disease and children with 11q loss or unfavourable histology needed the longer course. SIOPEN's LINES trial applies the same approach in Europe, and infants with stage MS disease who need treatment for a bulky liver receive the same drugs briefly.
| Setting | Approach | Guideline |
|---|---|---|
| Chemotherapy | Two to eight cycles of carboplatin, etoposide, cyclophosphamide and doxorubicin by biology and response (A3961, ANBL0531), then surgery when resectable. | not mapped |
| Response assessment and surgery | MRI or CT and MIBG scan after chemotherapy; resection of the residual primary where safe, otherwise observation of the residual mass. | not mapped |
| Non-responding or life-threatening disease | Radiotherapy to the primary or to a compressing lesion; escalation to high-risk therapy if biology proves unfavourable. | not mapped |
| Follow-up | Hearing, kidney, cardiac and fertility follow-up for the chemotherapy given. | not mapped |