The first 60 days: Intermediate-risk neuroblastoma

Below, week by week, is what OnCo's record of Intermediate-risk neuroblastoma says about the first two months: the order is typical, the timing is yours to ask about. 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. 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 (L2, M in infants, MS)MYCN amplification (must be absent)11q aberration DNA ploidy INPC histology ALK mutation Urinary catecholamine metabolites

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

MRI MIBG imaging and 131I-MIBG therapy

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.Response assessment and surgery NCI PDQ: Neuroblastoma Treatment (health professional version)
MRI or CT and MIBG scan after chemotherapy; resection of the residual primary where safe, otherwise observation of the residual mass.
MRI MIBG imaging and 131I-MIBG therapy INRG staging and risk groups
2.Chemotherapy NCI PDQ: Neuroblastoma Treatment (health professional version)
Two to eight cycles of carboplatin, etoposide, cyclophosphamide and doxorubicin by biology and response (A3961, ANBL0531), then surgery when resectable.
Carboplatin Etoposide Cyclophosphamide Doxorubicin Cytotoxic chemotherapy INRG staging and risk groups ANBL0531
3.Non-responding or life-threatening disease NCI PDQ: Neuroblastoma Treatment (health professional version)
Radiotherapy to the primary or to a compressing lesion; escalation to high-risk therapy if biology proves unfavourable.
IMRT / IGRT (modern external beam)Radiotherapy
4.Follow-up NCI PDQ: Neuroblastoma Treatment (health professional version)
Hearing, kidney, cardiac and fertility follow-up for the chemotherapy given.
Late effects and survivorship toxicity Cardio-oncology Oncofertility and fertility preservation

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 stage, 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 L2 neuroblastoma over 18 months, or with unfavourable histology or 11q aberration, without MYCN amplification, Stage M neuroblastoma in infants under 18 months without MYCN amplification, Stage MS neuroblastoma with 11q aberration or unfavourable biology.
Is germline (inherited) genetic testing recommended for me or my family?Inherited variants can change treatment and matter for relatives.

Chemotherapy

For my situation (chemotherapy), which of the standard options do you recommend and why?Guideline options include: Two to eight cycles of carboplatin, etoposide, cyclophosphamide and doxorubicin by biology and response (A3961, ANBL0531), then surgery when resectable.
Am I a candidate for Carboplatin, Etoposide, Cyclophosphamide or related drugs, and what side effects should I expect?Knowing the expected toxicities helps you plan work, family, and supportive care.
How do the results of ANBL0531 apply to someone like me?Trial populations differ from individual patients; ask how closely you match.

Response assessment and surgery

For my situation (response assessment and surgery), which of the standard options do you recommend and why?Guideline options include: MRI or CT and MIBG scan after chemotherapy; resection of the residual primary where safe, otherwise observation of the residual mass.

Non-responding or life-threatening disease

For my situation (non-responding or life-threatening disease), which of the standard options do you recommend and why?Guideline options include: Radiotherapy to the primary or to a compressing lesion; escalation to high-risk therapy if biology proves unfavourable.

Follow-up

For my situation (follow-up), which of the standard options do you recommend and why?Guideline options include: Hearing, kidney, cardiac and fertility follow-up for the chemotherapy given.

Any stage

Are there clinical trials I could join, for example of INRG staging and risk groups, 18F-MFBG PET replacing 123I-MIBG scintigraphy, MIBG imaging and 131I-MIBG therapy, Cardio-oncology?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 “Children with 11q aberration or unfavourable histology still relapse and cannot have therapy shortened”. How does that affect my plan?Open problems are where trials and second opinions matter most.
I read that “Whether telomere maintenance and ALK status should change risk assignment”. 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.
Radiotherapy: Using high-energy X-rays or particles to damage the DNA of cancer cells in a precisely aimed volume of the body.
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.