Cancer Center

Relapsed or Refractory Neuroblastoma in Children

What is relapsed or refractory neuroblastoma?

While low-risk and intermediate-risk forms of neuroblastoma may regrow (relapse) after surgery or chemotherapy, these children are usually cured with standard techniques such as surgery or chemotherapy. However, for about 10 percent of children with high-risk neuroblastoma the disease will not respond to chemotherapy (refractory). In another 40 to 50 percent of cases the disease will go away at some point but eventually relapse during or after treatment.

Symptoms of relapsed or refractory neuroblastoma

Just like newly diagnosed neuroblastoma, signs and symptoms of a relapse will vary depending on the site of the primary tumor or and how much it has spread (metastasized). Relapse is often detected on scans when there are no symptoms at all. Pain is the most common sign of relapse, but there are many symptoms ranging from fatigue to new areas of swelling.

Diagnosing relapsed or refractory neuroblastoma

Your child will have regularly scheduled scans for about 3 years after initial treatment, which will alert your child’s medical team of a potential relapse very quickly. If a relapse is suspected, your child will undergo a series of tests to show the extent of the relapse. Some of the tests your child might undergo include:

Treatment for relapsed neuroblastoma

Children with relapsed or refractory neuroblastoma are treated by the Refractory Neuroblastoma Program. This program is staffed by a team of neuroblastoma experts who will help your family decide the next best step in your child’s treatment.

Recommendations for management of relapsed neuroblastoma are made based upon the individual characteristics of the patient and his or her tumor. Standard chemotherapy such as irinotecan and temozolomide may be used as a bridge to other treatments. CHOP has the largest targeted radiotherapy program for neuroblastoma in the world, and I-131-MIBG is commonly used either alone or in combination with other drugs to reduce tumor burden.

I-131 MIBG is a drug that is used to treat neuroblastoma. It is a clear liquid, made of two materials: metaiodobenzylguanidine, or MIBG, and I-131, a radioactive material. The MIBG carries the radioactive I-131 specifically to your child’s tumors, where it targets the cancer cells over many weeks. A significant amount of the radioactive material is filtered into the kidneys, and passed out of the body through your child’s urine over several days.

To limit radiation exposure to others, you will be the primary caregiver for your child, who will stay in a special room at the hospital until the radiation has gone down to a safe level. Learn more about the MIBG experience, and access resources specifically for your child.

Clinical trials

CHOP is a member of all major pediatric cancer clinical trial networks, most importantly the Children’s Oncology Group (COG) Phase 1 consortium and the New Approaches to Neuroblastoma Therapy (NANT) consortium. Our goal is to maximize the number of Phase 1 and Phase 2 clinical trials available for children with neuroblastoma. Our goal is to match an individual patient to the best available clinical trial that is most likely to reduce the amount of cancer, with minimal side effects.

For example, for a child whose tumor has a mutation in the ALK protein, a clinical trial with a specific inhibitor of this protein may be offered. Our team knows the nuances of what makes a child a good candidate for this or other therapies. There are an evolving series of new clinical trials, many developed here at CHOP, that may be recommended for any particular child.

Before beginning any clinical trial, your family will have a consultation with the Refractory Neuroblastoma Program to determine if your child is eligible. After consultation, the team designs and delivers the best possible therapies, many of which are experimental, for each child as his situation with the disease evolves.

Working with the referring physician

If your child was initially treated at a different hospital, our neuroblastoma team will partner with the referring institution in the design and delivery of these new medicines. The refractory neuroblastoma team sees patients from around the country, and can see children from other countries when proper arrangements are made.

Long-term outlook

At this time, there is no known standard approach to cure relapsed high-risk neuroblastoma. The relapsed neuroblastoma team combines a very active laboratory research program with a major emphasis on translating new discoveries to the clinic with the goal of changing this reality.

Currently, experts can personalize the approach to relapsed or refractory high-risk neuroblastoma with the goal of achieving a remission while maintaining outstanding quality of life. The team is aggressive in seeking new treatment options proactively, and using the most up-to-date information to support decision-making.

Relapsed neuroblastoma team


Nurse practitioners

Research nurse

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