Neuroblastoma

Here at Dana-Farber/Children's Hospital Cancer Center, we know how difficult a diagnosis of neuroblastoma can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child. 

What are the treatments for neuroblastoma?

Your child’s physician will determine a specific course of treatment for neuroblastoma based on several factors, including:

• the extent of the disease
• the size and location of the tumor
• the tumor's characteristics when examined under a microscope
• the presence or absence of metastasis
• the tumor's response to therapy
• the age and overall health of your child
• your child's tolerance of specific medications, procedures, or therapies
• new developments in treatment

Treatment of this tumor requires extremely close coordination among the pediatric oncologists, radiotherapists and surgeons.

This tumor, more than any other, has a wide spectrum of behavior.

• Neuroblastomas arising in children under age 1 often have very slow progression and are readily treated.
• Those occurring in children over one year of age are often more aggressive and more difficult to cure.
  • These tumors may be quite extensive at the time of presentation and those in the abdomen often surround the blood vessels to the kidney as well as the blood vessels to the liver, spleen and small intestine.

There are a number of treatments we may recommend. Some of them help to treat the tumor while others are intended to address complications of the disease or side effects of the treatment.

Low- and intermediate-risk neuroblastoma

If your child has a small tumor, our pediatric surgeons will remove the tumor and determine whether the tumor has spread. The tumor specimen will then be analyzed by our pediatric pathologist. The information gathered will help your child’s physician determine the stage of neuroblastoma and decide which additional therapy will be used. Low risk tumors in young children are often cured with surgery alone.

Your child’s physician may also recommend chemotherapy in some cases of low risk neuroblastoma and most cases of intermediate risk neuroblastoma.

High risk neuroblastoma

If your child has a large tumor and is over the age of one, surgeons will perform an initial biopsy. Your child will then receive chemotherapy and, sometimes, radiation therapy prior to removal of the tumor.

Initial treatment with chemotherapy provides several advantages:

• It often produces a dramatic decrease in the size of the tumor, making it easier for our surgeons to remove completely.
• It generally decreases the chances of rupture of the tumor or significant bleeding during the surgery.
• Separation of the tumor from the critical blood vessels it surrounds can be accomplished best in tumors treated with chemotherapy.

If your child has a tumor that has spread to the bone marrow or bones and has additional risk factors (including being over 1 year of age and having genetic findings suggesting an aggressive tumor), your child’s physician may recommend a stem cell transplant. This procedure is performed after chemotherapy and surgical removal of the tumor to enhance your child’s chances of cure.

We may also recommend radiation therapy. Radiation therapy is used in areas where the tumor has been incompletely removed, in tumors with expected aggressive behavior, and in conjunction with stem cell transplantation.

We may also recommend targeted antibody therapy to treat high-risk neuroblastoma. This therapy uses specialized antibodies to target and kill the neuroblastoma cells directly, sparing health tissues.

Neuroblastoma in infants

In very young children, neuroblastoma sometimes does not require treatment. If the tumor is very small, your child may be treated with observation alone. Surgery is performed later on if the tumor does not go away on its own.

In addition, a specific type of metastatic neuroblastoma, called 4S, can also occur in infants. This type of neuroblastoma may have spread to the liver, skin and bone marrow. In some cases, these neuroblastomas may go away on their own and not require treatment.

Your child’s care team will determine whether he’ll require surgery and other therapies.

How is chemotherapy given?

Chemotherapy is a group of drugs that interfere with the cancer cell’s ability to grow or reproduce. Your child may receive chemotherapy:

• orally, as a pill to swallow
• intramuscularly, as an injection into the muscle or fat tissue
• intravenously, as a direct injection into the bloodstream; also called IV
• intrathecally, as a direct injection into the spinal column through a needle

Does chemotherapy cause side effects?

While chemotherapy can be quite effective in treating certain cancers, the medications cannot distinguish healthy cells from cancer cells. As a result, there can be side effects during treatment. Your child’s care team will work to manage or prevent these side effects when possible.

What treatments are available for relapsed neuroblastoma?

If you child’s neuroblastoma has recurred, he may receive a specialized treatment known as MIBG therapy.

• MIBG is a radioactive isotope that is readily absorbed by most neuroblastomas.
• MIBG therapy can be used to detect neuroblastoma in the body (MIBG scintiscan) or deliver radiation in order to kill the neuroblastoma cells.
• In general, about 40 percent of patients respond to MIBG therapy.
• At Children’s, we have a specialized MIBG room where children receive therapy. The room
• MIBG therapy takes one to two hours to administer.
• Because MIBG is radioactive, your child may need to stay in the hospital for three to four days until radiation levels decrease.
• The major side effect of therapy is low blood cell counts. In some cases, this may be treated with a stem cell transplant.
• We are currently investigating ways to improve the effectiveness of MIBG therapy further.

What is the long-term outlook for my child?

Your child’s prognosis (chance of recovery) and treatment options depend on a number of different factors:

• the extent of the disease
• the size and location of the tumor
• the tumor's characteristics when examined under a microscope
• the presence or absence of metastasis
• the tumor's response to therapy
• the age and overall health of your child
• your child's tolerance of specific medications, procedures, or therapies
• new developments in treatment

In general, low and intermediate risk neuroblastomas tend to be more treatable. High risk neuroblastomas are more difficult to treat and require more aggressive therapy. Prompt medical attention and appropriate therapy are important for the best prognosis.

Continual follow-up care is essential if your child has neuroblastoma. Side effects of radiation and chemotherapy, as well as recurrence of the disease, can occur in survivors of neuroblastoma.

What is the recommended long-term care for children treated for neuroblastoma? Chemotherapy and radiation therapy can cause damage to healthy tissues. As a result, your child may experience long-term side effects after treatment for neuroblastoma. Your child should visit a survivorship clinic every year to:

• manage disease complications
• screen for early recurrence of cancer
• manage late effects of treatment

For follow-up, your child will have yearly evaluations by our multidisciplinary team of pediatric sub-specialists. A typical follow-up visit may include some or all of the following:

• a physical exam
• laboratory testing
• imaging scans (such as MRI or CT)

Coping & support

We understand that you may have a lot of questions if your child is diagnosed with neuroblastoma. What is the likelihood of cure? What do we do next? We’ve tried to provide some answers to those questions in these condition pages, but there are also a number of other resources to help you and your family through this difficult time.
 
Patient education: From the first visit through follow-up care, our nurses will be on hand to walk you through your child’s treatment and help answer any questions you may have — How long will my child be in the hospital? How often will my child require follow-up? They will also reach out to you by phone, continuing the care and support you received while at Children’s.
 
Parent to parent: Want to talk with someone whose child has been treated for a neuroblastoma? We can put you in touch with other families who have been through the same experience that you and your child are facing, and share with you their experience at Children’s.
 
Faith-based support: If you are in need of spiritual support, we will help connect you with the Children’s chaplaincy. Our program includes nearly a dozen clergy representing Episcopal, Jewish, Lutheran, Muslim, Roman Catholic, Unitarian and United Church of Christ traditions who will listen to you, pray with you and help you observe your own faith practices during your hospital experience.
 
Social work: Our clinical social workers have helped many other families in your situation. Your social worker can offer counseling and assistance with issues such as coping with your child’s diagnosis, stresses relating to coping with illness and dealing with financial difficulties.

Click here for more information on our pediatric support services.

Long-term follow-up

Many children diagnosed with neuroblastoma will survive into adulthood. However, survivors may face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

Through our David B. Perini, Jr. Quality of Life Clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

• Our childhood cancer survivorship clinic is held weekly.
• In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
• We also offer the following services:
  • patient and family education
  • psychosocial assessment
  • genetic counseling
  • reproductive and fertility evaluation and counseling
  • opportunities to speak with other childhood cancer survivors

Click here for more information on our pediatric support services.