Watch: the CureRays® explainer series
Prefer to read? The full guide below is complete on its own, with tables you can revisit any time.
What is neuroblastoma (childhood nerve cancer)?
Neuroblastoma is a cancer that develops from neuroblasts — immature nerve cells left over from a baby's development. It is one of the most common cancers in infants and young children, usually diagnosed before age 5, and rarely occurs in older children or adults. Because these early nerve cells are part of the sympathetic nervous system, neuroblastoma can start in several places: most often in the adrenal glands on top of the kidneys, but also along the chain of nerve tissue beside the spine in the abdomen, chest, neck, or pelvis. Symptoms depend on where the tumor is and whether it has spread — a child may have a swollen belly or a lump, bone pain, fever, fatigue, bruising around the eyes, or other signs. Neuroblastoma behaves very differently from child to child: some tumors, especially in babies, can mature or even disappear on their own with little or no treatment, while others are aggressive and need intensive therapy. Because of this wide range, doctors carefully sort each child into a risk group that guides treatment. Care is delivered by a specialized children's cancer team, and radiation therapy — including a special form that seeks out neuroblastoma cells throughout the body — is an important tool, especially for high-risk disease.
The main types
Doctors group neuroblastoma (childhood nerve cancer) by where it starts and how it behaves:
| Type | What it means, simply |
|---|---|
| Low-risk neuroblastoma | Often seen in infants; these tumors may shrink or mature on their own and frequently need only surgery or close observation, with excellent outcomes. |
| Intermediate-risk neuroblastoma | Treated with surgery and a moderate amount of chemotherapy; most children do well. |
| High-risk neuroblastoma | Aggressive disease, often spread by the time it is found; treated with intensive combined therapy including chemotherapy, surgery, radiation, stem-cell transplant, immunotherapy, and a special targeted radiation. |
| Stage MS (special infant form) | A unique pattern in babies where the cancer has spread to the skin, liver, or marrow but often regresses with little treatment and has a very good outlook. |
Staging, in plain terms
Neuroblastoma uses its own system rather than the TNM staging used for adult cancers. The INRG system describes the tumor as L1 (a localized tumor that is easy to remove), L2 (a localized tumor with features that make removal riskier, such as wrapping around blood vessels), M (cancer that has spread to distant sites), or MS (the special infant pattern of spread that often regresses). Just as important, doctors combine this stage with other factors — the child's age, how the cells look under the microscope, and genetic features of the tumor such as whether a gene called MYCN is amplified — to place each child into a low-, intermediate-, or high-risk group. This risk group, more than the stage alone, determines how intensive treatment needs to be, which is why two children with the same stage can receive very different plans.
| International Neuroblastoma Risk Group (INRG) stages L1, L2, M, MS — combined with a risk group (not TNM) | What it generally means |
|---|---|
| Stage L1 | A localized tumor confined to one area without risky features, usually removable with surgery alone. |
| Stage L2 | A localized tumor with features that make surgery riskier, such as growth around major blood vessels; may need chemotherapy to shrink it before removal. |
| Stage M | Cancer that has spread to distant parts of the body such as bones, bone marrow, or distant lymph nodes; usually high-risk and treated intensively. |
| Stage MS | A special form in infants with spread to the skin, liver, or limited marrow; it often regresses with little or no treatment and has a favorable outlook. |
The standard of care
Neuroblastoma (Childhood Nerve Cancer) is almost always treated by a team that may include a surgeon, a medical oncologist, and a radiation oncologist, combining therapies for the best result. The usual building blocks are:
Observation
Some low-risk tumors, especially in infants, are watched closely because they can mature or shrink on their own without treatment.
Surgery
Removing the tumor is a key step for localized disease and part of treatment for higher-risk disease after chemotherapy has shrunk it.
Chemotherapy
Combination chemotherapy shrinks tumors and treats spread; the intensity is matched to the risk group.
Radiation therapy
Radiation to the tumor site and to areas of spread is an important part of high-risk treatment, helping prevent the cancer from coming back; a special targeted radiation (MIBG) can also seek out neuroblastoma cells throughout the body.
Intensive therapy for high-risk disease
High-risk neuroblastoma is treated with a multi-step program that adds high-dose chemotherapy with a stem-cell transplant, immunotherapy, and a medicine called retinoic acid to lower the chance of relapse.
How radiation treatment works
Radiation uses focused high-energy beams to damage the DNA inside cancer cells so they can no longer grow and divide. Neuroblastoma is sensitive to radiation, so it is an important tool — but it is used selectively, mainly for high-risk disease, where radiation to the original tumor site and to areas of spread helps keep the cancer from coming back after chemotherapy and surgery. Because the patients are young children whose bodies are still growing, radiation oncologists plan treatment with great care to protect the spine, kidneys, liver, and other organs, sometimes using proton therapy to reduce the dose to healthy tissue. Neuroblastoma also has a special, almost unique option: MIBG therapy, a radioactive medicine given through a vein that is absorbed specifically by neuroblastoma cells throughout the body, delivering targeted radiation directly to the cancer wherever it hides. Standard radiation treatments are quick and painless, given over a small number of short daily sessions, and young children may have gentle sedation to help them stay still. Side effects are watched closely by the children's cancer team, with special attention to long-term growth and health. Your child's team will explain exactly where radiation fits in the overall plan.
The main ways radiation is delivered for neuroblastoma (childhood nerve cancer):
External-beam radiation to the tumor bed
Targeted radiation to the area where the tumor was removed treats any cancer cells left behind; it is carefully shaped to protect a young child's growing spine, kidneys, and other organs.
MIBG targeted radiation therapy
A radioactive medicine called MIBG is given through a vein and is taken up specifically by neuroblastoma cells throughout the body, delivering radiation directly to the cancer wherever it is — a form of theranostic, targeted radiation used in selected high-risk or relapsed cases.
Proton therapy
Proton beams deliver radiation to the target while reducing dose to growing bones and nearby organs, lowering the risk of long-term side effects in children.
Latest studies shaping care
Care keeps improving — often toward getting the same excellent results with less burden on patients. A few developments:
Immunotherapy improves high-risk outcomes: Adding antibody-based immunotherapy (anti-GD2) to treatment has improved survival for children with high-risk neuroblastoma and is now part of standard care after intensive initial therapy.[1]
High-risk neuroblastoma immunotherapy trials
MIBG targeted radiation: Radioactive MIBG, which homes in on neuroblastoma cells throughout the body, is being studied and used to treat high-risk and relapsed disease, delivering radiation precisely to the cancer.[2]
MIBG therapy clinical studies
Less treatment for low-risk children: Studies show that many low-risk tumors, especially in infants, can be safely observed or treated with minimal therapy because they mature or regress on their own, sparing young children unnecessary treatment.[3]
Low-risk neuroblastoma observation studies
Common questions
Can neuroblastoma be cured? Often, yes. Outcomes depend heavily on the risk group. Children with low- and intermediate-risk neuroblastoma usually do very well, and some infant tumors even shrink on their own. High-risk disease is more challenging, but intensive modern treatment — including chemotherapy, surgery, radiation, transplant, and immunotherapy — cures many children and continues to improve.
Why do two children with neuroblastoma get such different treatment? Neuroblastoma varies enormously from child to child. Doctors combine the tumor's stage with the child's age, how the cells look, and genetic features such as the MYCN gene to assign a risk group. A low-risk child may need only surgery or observation, while a high-risk child needs intensive combined therapy — which is why plans differ so much.
What is MIBG therapy? MIBG is a special targeted radiation treatment. It is a radioactive medicine given through a vein that neuroblastoma cells absorb specifically, delivering radiation directly to the cancer cells throughout the body wherever they are. It is used in selected high-risk or relapsed cases, and your child's team will explain whether it is part of the plan.
References
Numbered sources for the studies cited above. Links open the primary publication on PubMed or the publisher’s site.