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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.
Radiotherapy is used only for life-threatening disease that does not respond, and the residual mass after chemotherapy is often left in place when resection would risk the kidney or a nerve root. Children with 11q aberration or unfavourable histology, and infants with stage M disease and diploid tumours, are the ones who still relapse and the ones whose therapy the next trials will not shorten. Telomere maintenance mechanisms (ATRX, TERT) and ALK mutations are being tested as additions to the classification, and long-term follow-up of hearing, kidney function and fertility is what tells the groups whether the reductions were worth it.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
About one neuroblastoma in ten is intermediate risk: unresectable localised disease or metastatic disease in infants, without MYCN amplification, cured in about nine in ten children with a few cycles of moderate chemotherapy.
Renal cell carcinoma comes from the kidney's filtering cortex, urothelial cancer from the lining of the collecting system and bladder, and the adrenal on top hosts cortical and medullary (neuroblastoma) tumours.
Same organ: Collecting duct carcinoma of the kidney, Renal medullary carcinoma (SMARCB1-deficient), TFE3-rearranged (translocation) renal cell carcinoma, Fumarate hydratase-deficient renal cell carcinoma (HLRCC-associated), Succinate dehydrogenase-deficient renal cell carcinoma, Mucinous tubular and spindle cell carcinoma of the kidney, Eosinophilic solid and cystic renal cell carcinoma, Clear cell papillary renal cell tumour, Urothelial carcinoma of the urethra, Squamous cell carcinoma of the urethra, Adenocarcinoma of the urethra (including clear cell adenocarcinoma), Melanoma of the urethra, Non-muscle-invasive bladder cancer, Muscle-invasive and advanced bladder cancer, Bladder & urothelial cancer, Clear cell renal cell carcinoma, Papillary renal cell carcinoma, Chromophobe renal cell carcinoma, Renal cell carcinoma, Wilms tumour (nephroblastoma), Neuroblastoma (paediatric), Low-risk neuroblastoma (INRG very low and low risk, including stage MS), High-risk neuroblastoma, Adrenocortical carcinoma, Pheochromocytoma and paraganglioma (PPGL), Urethral cancer, Penile cancer, Localised penile cancer (organ-confined, node-negative), Node-positive and metastatic penile cancer, Localised adrenocortical carcinoma (ENSAT stage I to III, resectable), Advanced and metastatic adrenocortical carcinoma (ENSAT stage IV or unresectable), Hereditary pheochromocytoma and paraganglioma (SDHx, VHL, RET, NF1, MAX and TMEM127), Metastatic pheochromocytoma and paraganglioma
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Two to eight cycles of carboplatin, etoposide, cyclophosphamide and doxorubicin by biology and response (A3961, ANBL0531), then surgery when resectable.
MRI or CT and MIBG scan after chemotherapy; resection of the residual primary where safe, otherwise observation of the residual mass.
Radiotherapy to the primary or to a compressing lesion; escalation to high-risk therapy if biology proves unfavourable.
Hearing, kidney, cardiac and fertility follow-up for the chemotherapy given.
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Current intermediate-risk neuroblastoma protocols minimise chemotherapy to two to eight cycles by response and biology, following this study.
Biology-based reduction of chemotherapy is the standard for intermediate-risk neuroblastoma, and the successor trial ANBL0531 reduced it further.
Every neuroblastoma risk group on this site (very low, low, intermediate, high) is defined by the INRG system, which allows trials across continents to be compared.
Query for this cancer: (TITLE:"Intermediate-risk neuroblastoma" OR ABSTRACT:"Intermediate-risk neuroblastoma" OR TITLE:"INRG intermediate-risk neuroblastoma" OR ABSTRACT:"INRG intermediate-risk neuroblastoma" OR TITLE:"Unresectable localised neuroblastoma" OR ABSTRACT:"Unresectable localised neuroblastoma" OR TITLE:"Stage M neuroblastoma in infants" OR ABSTRACT:"Stage M neuroblastoma in infants") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Intermediate-risk neuroblastoma, not a curated reading list.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Dose by Calvert formula using GFR (see the calculators).
Reduce to 75% for CrCl 15-50.
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
Alkylating chemotherapy can damage a blood stem cell in a way that shows up years later as myelodysplastic syndrome or acute myeloid leukaemia. It is uncommon, it depends on the total dose, and the risk falls away after about ten years. Knowing the cumulative dose you were given is the single most useful thing on your treatment summary.
See all on the product pages:CarboplatinCyclophosphamideDoxorubicinEtoposide·Printable cards in the navigator
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Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record.
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