Low-risk neuroblastoma (INRG very low and low risk, including stage MS)
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.
Overview
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.
State of the art
- Surgery alone, or observation alone, cures almost every child with low-risk neuroblastoma; five-year overall survival was 97 percent in P9641.
- Small adrenal masses in young infants are observed without biopsy, and about half regress.
- Biology (MYCN, 11q, ploidy) rather than stage decides who is watched and who is treated.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Check before combiningKidneys: Carboplatin
Dose by Calvert formula using GFR (see the calculators).
- Check before combiningKidneys: Etoposide
Reduce to 75% for CrCl 15-50.
- Good to knowInfusion reactions, hypersensitivity and extravasation
Reactions around the moment a drug is given: chills, fever or breathlessness from antibodies (infusion reactions), true allergy (hypersensitivity, rarely anaphylaxis), and leakage of a damaging drug into tissue around the vein (extravasation).
See all on the product pages:CarboplatinCyclophosphamideEtoposide·Printable cards in the navigator
Anatomy and lymph node drainage
- Renal cortex (RCC)
- Renal pelvis and ureter (upper tract urothelial)
- Bladder lining (non-muscle-invasive)
- Bladder muscle wall (muscle-invasive)
- Adrenal cortex
- Adrenal medulla and sympathetic chain (neuroblastoma)
- Developing kidney (Wilms tumour)
- Nodes: renal hilar
- Nodes: para-aortic and paracaval
- Nodes: obturator and iliac (bladder)
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.
- Renal cortex (RCC)
- Renal pelvis and ureter (upper tract urothelial)
- Bladder lining (non-muscle-invasive)
- Bladder muscle wall (muscle-invasive)
- Adrenal cortexPerinatal adrenal neuroblastoma found on antenatal or postnatal ultrasound (expectant observation)
- Adrenal medulla and sympathetic chain (neuroblastoma)INRG very low risk: L1 neuroblastoma without MYCN amplification (surgery or observation) · INRG low risk: L2 neuroblastoma in infants without unfavourable biology (observation or surgery) · Stage MS neuroblastoma in infants under 18 months without MYCN amplification (observation, short chemotherapy if symptomatic) · Ganglioneuroblastoma intermixed and ganglioneuroma (maturing spectrum; surgery or observation) · Perinatal adrenal neuroblastoma found on antenatal or postnatal ultrasound (expectant observation)
- Developing kidney (Wilms tumour)
- renal hilar
- para-aortic and paracaval
- obturator and iliac (bladder)
Same organ: 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
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.
- MRIStandard of care
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
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.
Subtypes & biomarkers
top- INRG very low risk : L1 neuroblastoma without MYCN amplification (surgery or observation)
- INRG low risk : L2 neuroblastoma in infants without unfavourable biology (observation or surgery)
- Stage MS neuroblastoma in infants under 18 months without MYCN amplification (observation, short chemotherapy if symptomatic)
- Ganglioneuroblastoma intermixed and ganglioneuroma (maturing spectrum; surgery or observation)
- Perinatal adrenal neuroblastoma found on antenatal or postnatal ultrasound (expectant observation)
- INRG stage (L1, L2, MS) from image-defined risk factors
- MYCN amplification (must be absent)
- 11q aberration
- DNA ploidy
- INPC histology
- Urinary catecholamine metabolites (HVA, VMA)
- Age at diagnosis
How often this target appears
- 1971Evans staging of neuroblastoma introduced; stage IV-S recognised as regressing disease
- 2008German NB97: spontaneous regression in about half of observed infant tumours
- 2009International Neuroblastoma Risk Group classification published
- 2012P9641: surgery alone gives 97 percent survival in low-risk disease; expectant observation of small adrenal masses reported
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 5 changes by month →- 2026-09-17This recordLow-risk neuroblastoma (INRG very low and low risk, including stage MS)Facts on this page last checked
When this page itself was last checked or edited.
- 2012MilestoneActive surveillanceP9641: surgery alone gives 97 percent survival in low-risk disease; expectant observation of small adrenal masses reported
A milestone in how this cancer is treated.
- 2009MilestoneINRG staging and risk groupsInternational Neuroblastoma Risk Group classification published
A milestone in how this cancer is treated.
- 2008MilestoneLow-risk neuroblastoma (INRG very low and low risk, including stage MS)German NB97: spontaneous regression in about half of observed infant tumours
A milestone in how this cancer is treated.
- 1971MilestoneLow-risk neuroblastoma (INRG very low and low risk, including stage MS)Evans staging of neuroblastoma introduced; stage IV-S recognised as regressing disease
A milestone in how this cancer is treated.
What is in development for Low-risk neuroblastoma (INRG very low and low risk, including stage MS), drawn from the whole corpus: 1 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Ideas not yet in a trial · 1
Open problems and what is being done
Distinguishing tumours that will regress from those that will grow without biopsy.
When surgery to the kidney or spinal canal is riskier than watching.
Identifying the few stage MS infants with MYCN amplification or 11q loss who behave as high risk.
Trials
topTrials recruiting now
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Expert centres
topExpert centres
Ljubljana · cancer center | Slovenia | none recorded | 0 | 187 | 2,566 | - | |
Neu-Isenburg · consortium | Germany | none recorded | 0 | 94 | 1,947 | none recorded | - |
Chicago, IL · consortium | United States | none recorded | 0 | 42 | 482 | - | |
Monrovia, CA · consortium | United States | none recorded | 0 | 20 | 186 | none recorded | - |
Brussels · consortium | Belgium | none recorded | 0 | 7 | 76 | none recorded | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Low-risk neuroblastoma but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Low-risk neuroblastoma
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example INRG stagefrom image-defined risk factors, MYCN amplification, 11q aberration, DNA ploidy, INPC histology), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include INRG very low risk: L1 neuroblastoma without MYCN amplification, INRG low risk: L2 neuroblastoma in infants without unfavourable biology, Stage MS neuroblastoma in infants under 18 months without MYCN amplification.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
L1 tumours and small adrenal masses in infants
- For my situation (l1 tumours and small adrenal masses in infants), which of the standard options do you recommend and why?Why: Guideline options include: Observation with serial ultrasound and urinary catecholamines, or surgical resection; no chemotherapy.
L2 tumours in infants with favourable biology
- For my situation (l2 tumours in infants with favourable biology), which of the standard options do you recommend and why?Why: Guideline options include: Expectant observation or surgery; short carboplatin and etoposide or cyclophosphamide only for symptoms or threat to organs.
- Am I a candidate for Carboplatin, Etoposide, Cyclophosphamide, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Stage MS
- For my situation (stage ms), which of the standard options do you recommend and why?Why: Guideline options include: Observation; carboplatin and etoposide or low-dose cyclophosphamide for a rapidly enlarging liver or respiratory compromise.
- Am I a candidate for Carboplatin, Etoposide, Cyclophosphamide, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Follow-up
- For my situation (follow-up), which of the standard options do you recommend and why?Why: Guideline options include: Ultrasound and urinary catecholamines, tapering over years; late-effects follow-up for the few who received chemotherapy.
Any stage
- Are there clinical trials I could join, for example of Active surveillance, INRG staging and risk groups, 18F-MFBG PET replacing 123I-MIBG scintigraphy, Ultrasound?Why: Trials are how the next standard of care is set; asking early keeps options open.
- Would a second opinion at a high-volume centre change anything, and can you help arrange it?Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
- What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?Why: Supportive care improves quality of life and helps patients complete treatment.
- I read that “Distinguishing tumours that will regress from those that will grow without biopsy”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “When surgery to the kidney or spinal canal is riskier than watching”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Low-risk neuroblastoma, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
9targets
1drugs
3institutions
2terms
5ideas
1Latest papers
topQuery 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.
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