The first 60 days: Low-risk neuroblastoma (INRG very low and low risk, including stage MS)

Below, week by week, is what OnCo's record of Low-risk neuroblastoma (INRG very low and low risk, including stage MS) says about the first two months: the order is typical, the timing is yours to ask about. 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. Sections appear only where the record has something to say. Orientation, not medical advice.

Weeks 1 to 2

What happens now

Staging tests establish exactly what and where the cancer is. Everything else follows from the answers.

Biomarkers your report may list

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

Ask which of these were tested and what the results were; see the report reader for what each value means.

Tests and imaging linked to this cancer

Active surveillance MRI Ultrasound

Weeks 1 to 3

Who is on your team

These specialties appear in the standard of care for this cancer. In most centres they meet weekly as a tumour board to agree each plan; you can ask when yours was discussed and what was decided.

A second opinion from a centre that treats many similar cases is normal, not rude; the standard of care tells you what to compare it against.

Weeks 2 to 6

Decisions coming up

Each row is a setting from the standard of care, in the order it usually arises. Not all will apply to you; your stage and biomarkers decide which do. The guideline grade, where recorded, says how strong the evidence is.

1.L1 tumours and small adrenal masses in infants NCI PDQ: Neuroblastoma Treatment (health professional version)
Observation with serial ultrasound and urinary catecholamines, or surgical resection; no chemotherapy.
INRG staging and risk groups Active surveillance Ultrasound MRI MYCN amplification
2.L2 tumours in infants with favourable biology NCI PDQ: Neuroblastoma Treatment (health professional version)
Expectant observation or surgery; short carboplatin and etoposide or cyclophosphamide only for symptoms or threat to organs.
Active surveillance Carboplatin Etoposide Cyclophosphamide INRG staging and risk groups
3.Stage MS NCI PDQ: Neuroblastoma Treatment (health professional version)
Observation; carboplatin and etoposide or low-dose cyclophosphamide for a rapidly enlarging liver or respiratory compromise.
Active surveillance Carboplatin Etoposide Cyclophosphamide Ultrasound
4.Follow-up NCI PDQ: Neuroblastoma Treatment (health professional version)
Ultrasound and urinary catecholamines, tapering over years; late-effects follow-up for the few who received chemotherapy.
Ultrasound MRI Late effects and survivorship toxicity

Every visit

Questions to ask at each visit

Generated from this cancer's standard of care, biomarkers and open problems. Take the group that matches where you are. To tick, add your own and print, open the one-page appointment sheet.

Newly diagnosed

What is my exact diagnosis, stage, and grade, and which tests established them?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?These results decide eligibility for targeted therapy, immunotherapy, and trials.
Which subtype is my cancer, and does that change the recommended treatment?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?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?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?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?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?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?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?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?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?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?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?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?Open problems are where trials and second opinions matter most.

From day one

Help that costs nothing

Much of what helps in the first weeks is free if you know to ask: testing, helplines, rides and lodging, second opinions, trial travel.

Any time

What to read next

Words this page and the cancer page use

INRG staging and risk groups: INRG staging is the international system that sorts neuroblastoma into four risk groups, from the lowest (often observed, sometimes regressing on its own) to high risk (about half of patients), using age under 18 months, spread, MYCN amplification, 11q status, ploidy and histology.
MYCN amplification: Extra copies of the MYCN oncogene, found in about 20% of neuroblastomas, mark the most aggressive disease and define high risk at any age.
Segmental chromosomal aberrations and ploidy (neuroblastoma): In a child's neuroblastoma the pattern of chromosome damage is a crystal ball: tumours that have gained or lost whole chromosomes tend to regress or be cured, tumours with broken segments (11q loss, 1p loss, 17q gain) or with MYCN amplified relapse, and the pattern moves a child between low, intermediate and high-risk treatment.
Urinary catecholamine metabolites (VMA and HVA): Neuroblastoma cells make adrenaline-type hormones and spill their breakdown products, VMA and HVA, into urine; a simple urine test supports the diagnosis in nine out of ten children, and falling levels after treatment or rising ones in follow-up track the tumour without a scan.
Late effects and survivorship toxicity: Health problems appearing months or decades after treatment ends: heart damage, infertility, second cancers, lymphoedema, dry mouth, memory problems, weak bones.

Every term links to the glossary.