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Low-risk neuroblastoma is the form found in infants and young children whose tumour has not spread beyond its site or, in the special stage MS pattern, has spread only to the liver, skin and a little marrow. Many of these tumours shrink and disappear on their own, so treatment is surgery, or simply watching, and almost every child survives.
Neuroblastoma arises from sympathetic nervous system precursors, most often in the adrenal, and the same histology spans one of the widest ranges of behaviour in oncology. The International Neuroblastoma Risk Group (INRG) classification of 2009 assigns pretreatment risk from image-defined risk factors (stage L1 or L2), metastatic pattern (M or MS), age, MYCN status, 11q aberration, ploidy and histology. Very low and low risk covers L1 tumours without MYCN amplification at any age, L2 tumours in infants without unfavourable biology, and stage MS in infants under 18 months: metastases confined to skin, liver and less than 10 percent of marrow, which regress spontaneously. Screening programmes in Japan, Quebec and Germany in the 1980s and 1990s found many more infant tumours than ever came to clinical attention and did not reduce deaths, proof that a large fraction of infant neuroblastoma regresses unseen.
The Children's Oncology Group trial P9641 treated children with low-risk disease by surgery alone, reserving chemotherapy for symptoms or incomplete resection with unfavourable biology: five-year event-free survival was 89 percent and overall survival 97 percent, with almost every child who relapsed rescued. The German NB97 trial observed infants with localised unresected tumours and saw spontaneous regression in around half. COG ANBL1232 then went further, observing small adrenal masses in infants under six months without biopsy, and expectant observation of L2 tumours in children under 18 months with favourable biology; the SIOPEN LINES study runs the same strategy in Europe. Stage MS infants are watched unless a rapidly enlarging liver threatens breathing or the kidneys, when a short course of carboplatin and etoposide or low-dose cyclophosphamide is given.
The problems are of judgement rather than drugs: telling a tumour that will regress from one that will grow, deciding when the surgical risk to the kidney or spinal cord of an L2 tumour outweighs the risk of watching, and recognising the minority of infants with MS disease who carry MYCN amplification or 11q loss and behave as high risk. Telomere maintenance status and segmental chromosomal aberrations are being added to the biology, urinary catecholamines and ultrasound carry the follow-up, and the late effects of the chemotherapy given to the small group who need it, particularly hearing and fertility, are being tracked so that even that can be reduced.
Roughly a third of neuroblastoma, mostly in infants, is low risk: localised disease or the special metastatic pattern of infancy, without MYCN amplification, and cured in almost every case with little or no treatment.
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), Intermediate-risk neuroblastoma, 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
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Also on OnCo: Symptoms and red flags · Early detection roadmap.
Observation with serial ultrasound and urinary catecholamines, or surgical resection; no chemotherapy.
Expectant observation or surgery; short carboplatin and etoposide or cyclophosphamide only for symptoms or threat to organs.
Observation; carboplatin and etoposide or low-dose cyclophosphamide for a rapidly enlarging liver or respiratory compromise.
Ultrasound and urinary catecholamines, tapering over years; late-effects follow-up for the few who received chemotherapy.
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Observation without biopsy is standard for small adrenal masses in young infants, sparing them surgery for tumours that often regress.
Surgery alone, or observation, is standard for low-risk neuroblastoma, with chemotherapy limited to those with symptoms or unfavourable biology.
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:"Low-risk neuroblastoma" OR ABSTRACT:"Low-risk neuroblastoma" OR TITLE:"INRG very low and low risk, including stage MS" OR ABSTRACT:"INRG very low and low risk, including stage MS" OR TITLE:"Very low-risk neuroblastoma" OR ABSTRACT:"Very low-risk neuroblastoma" OR TITLE:"Stage MS neuroblastoma" OR ABSTRACT:"Stage MS neuroblastoma" OR TITLE:"Stage 4S neuroblastoma" OR ABSTRACT:"Stage 4S neuroblastoma" OR TITLE:"Localised neuroblastoma in infants" OR ABSTRACT:"Localised neuroblastoma in infants") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Low-risk neuroblastoma (INRG very low and low risk, including stage MS), 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.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Dose by Calvert formula using GFR (see the calculators).
Reduce to 75% for CrCl 15-50.
The leukaemia risk after chemotherapy was described in the era of mustards and etoposide, and it did not stay there. Platinum drugs carry it, PARP inhibitors raise it about two and a half times against placebo, and lenalidomide with oral melphalan raises it nearly fivefold against melphalan alone. The absolute numbers are small, but the choice of partner drug is sometimes a real decision.
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.
Cyclophosphamide is one of the few cancer drugs that has been shown to cause a specific solid cancer, in the bladder, and the risk depends steeply on the total dose given. Blood in the urine years after treatment with it is a reason to be investigated rather than reassured, and the cumulative dose on your treatment summary is what tells you where you sit.
See all on the product pages:CarboplatinCyclophosphamideEtoposide·Printable cards in the navigator
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