About Neuroblastoma

Did you know?
  • Neuroblastoma has the highest mortality rate of all pediatric cancers.
  • Neuroblastoma is a common and often difficult to treat cancer, the most common cancer in infancy.
  • In the United States, about 650 children are diagnosed with neuroblastoma each year.
  • It is the most common tumor found in children younger than 1 year of age.
  • Neuroblastoma is the most common extra cranial solid tumor cancer in children.
  • Every 16 hours a child with neuroblastoma dies.
  • There is no known cure for relapsed neuroblastoma.
  • Nearly 70% of those children first diagnosed with neuroblastoma have disease that has already metastasized or spread to other parts of the body. When disease has spread at diagnosis and a child is over the age of 2, there is less than a 30% chance of survival.


46 children a day are diagnosed with cancer.
7 kids die every day from cancer.

Did you know?

  • Of the $9 billion dollars of government funding for cancer research, only 3% goes towards pediatric cancer.
  • Of that 3%, only approximately $150,000.00 goes towards Neuroblastoma research annually.
  • Juliana's autologous bone marrow transplant (also referred to as myeloblative or consolidation therapy) cost $1,465,000.00. She is one of 5 kids that we met in less than a year that needed a stem cell transplant, and the numbers of children that still need a bone marrow transplant are immeasurable.
What is Neuroblastoma?

Neuroblastoma is a tumor of nerve tissue that develops in infants and children and can occur in many areas of the body. It develops from the tissues that form the sympathetic nervous system (which controls body functions, such as heart rate and blood pressure, digestion and levels of certain hormones). It most commonly begins in the abdomen in the tissues of the adrenal gland, but it may also occur in other areas. It can spread to the lymph nodes, liver, bones and bone marrow. Juliana's tumor originated in her left kidney, causing her to be hypertensive. It never metastasized, but it was also found around her aorta, Celiac and SMA arteries and began to wrap around the blood vessel of her right kidney. 

Neuroblastoma may be considered a malignant manifestation of aberrant sympathetic nervous system development. Until recently, however, little was known about the genetic basis of this disease. As has been shown for many human cancers, a subgroup of cases display autosomal dominant inheritance. It was recently reported that activating mutations in the tyrosine kinase domain of the ana-plastic lymphoma kinase (ALK) oncogene account for most cases of hereditary neuroblastoma. These germline mutations encode for single-base substitutions in key regions of the kinase domain and result in constitutive activation of the kinase and a premalignant state. Mutations resulting in oncogene activation are also somatically acquired in 5 to 15% of neuroblastomas.

Who can be diagnosed with Neuroblastoma?

Neuroblastoma is typically diagnosed in babies or young children. The average age for neuroblastoma is about 18 months of age but it is occasionally seen in teenagers or even young adults. It is found slightly more often in boys than in girls. In the United States, about 650 new cases of neuroblastoma are diagnosed each year. It has rapidly progressed in the last 5 years.

What are the signs and symptoms of Neuroblastoma?

Signs and symptoms will vary depending on the site of the primary tumor or the extent that it has spread (metastasized). The most common primary site is the abdomen. Other primary sites include the chest, neck and pelvis. Neuroblastoma can spread to other areas of the body. If your child has a primary tumor in the abdomen, you may notice bellyaches, weight loss or a bigger abdomen. Some children only complain of general aches and pains. In Juliana's case, her symptoms were loss of appetite, hypertension, rapid weight loss and lethargy. If you think something just "isn't right" with your child - get it checked out immediately.

How is Neuroblastoma "officially" diagnosed?


Your child will need to have a series of tests to help learn about the extent of the disease. Some of the neuroblastoma tests include:

  • Urine VMA/HVA test. This test will determine the amount of a substance called catecholamines in the urine; the tumor secretes and produces catecholamines.
  • CT scan (computerized tomography) or MRI scan (magnetic resonance imaging). These scans are done to get pictures of the head, chest, abdomen and/or pelvis to measure the size of the tumor and to look for the spread of the disease.
  • Bone marrow aspirate and biopsy. Bone marrow is removed from the bone (usually the hip bone) either by aspiration (suctioning a small amount through a hollow needle) or by biopsy (cutting out a small piece of bone marrow). This test is generally done under anesthesia.
  • 123I-MIBG scan. A small amount of radioactive iodine (123I), linked to the very specific neuroblastoma chemical MIBG, is injected and an area of the body is viewed through pictures taken 24 hours later.
  • Surgical biopsy. This test allows us to collect a small sample of the tumor. It is done in the operating room under general anesthesia. After the surgery, a large panel of laboratory and genetic tests are done on the tumor sample to determine how aggressive it is. These tests include, but are not limited to, International Neuroblastoma Criteria grade, MYCN amplification status, DNA index, whole genome SNP array, ALK mutation and amplification status, and 1p and 11p deletion status.

We do not expect you to know what these tests mean, but your doctors use this information to determine the best treatment for your child.

What are the "stages" of Neuroblastoma?

The tests used to diagnose neuroblastoma can also be used to determine the stage of the disease. Stage is a term that refers to the location and extent of cancer tumor(s) and cells. Treatment of neuroblastoma can differ widely depending on the stage. The tumor stages outlined below are based on a tumor-staging system used by experts worldwide.

Stage 1
The tumor is confined to the area in which it arose (i.e., there has been no spread, or metastasis) and can be completely removed by surgery.

Stage 2A
The tumor is confined to the area in which it arose, but cannot be completely removed by surgery.

Stage 2B
The tumor is confined to the area in which it arose. It may or may not be possible to completely remove the tumor by surgery. Neuroblastoma cells are present in the lymph nodes adjacent to the tumor.

Stage 3
The tumor cannot be completely removed by surgery and has either:
- spread from one side of the body to the other and may also have spread to nearby lymph nodes;

- remained confined to the area in which it arose, but spread to lymph nodes on the other side of the body; or
- remained confined to the middle of the body and spread to lymph nodes on both sides of the body.

Stage 4
The tumor has spread to distant lymph nodes, bone marrow, bone, liver, or other organs (except as defined by stage 4S).

Stage 4S
Stage 4S is applicable only to children who are younger than one year. The tumor is confined to the area in which it arose, as in stages 1 and 2, but some cells have spread to the liver, skin, or bone marrow.


How is newly diagnosed Neuroblastoma treated?

You'll need to determine whether the disease risk is low, intermediate or high. 

  • Low-risk disease. If your child is under a year of age at diagnosis, or has small, easily removed tumors, the child may only need a simple surgical procedure or careful follow-up.
  • Intermediate-risk disease. If your child has a larger tumor that has not spread, or is a baby with disease that has spread (but does not have the MYCN abnormality mentioned above), they will probably treat your child with surgery and four to eight months of chemotherapy. Often, patients with intermediate-risk disease do not need radiation treatments.
  • High-risk disease. About half of neuroblastoma patients have high-risk disease, either because the tumor has spread to other parts of the body or because test results show high-risk features, such as MYCN amplification. If your child has high-risk neuroblastoma, treatment includes five to six months of chemotherapy, surgery, radiation therapy, high dose chemotherapy followed by his or her own stem cell rescue, and immunotherapy combined with biological therapy.

Newly diagnosed patients are generally treated according to Children's Oncology Group protocols. However, every child will receive individualized treatment planning and other treatments may be recommended.


How is relapsed or refractory neuroblastoma treated?

Unfortunately, high-risk neuroblastoma still has a very high rate of non-responsiveness, or recurring/relapsing during or after treatment. There is no standard treatment for recurrent neuroblastoma in patients first treated for high-risk neuroblastoma. Patients may want to consider a clinical trial. For information about clinical trials, please visit our Clinical Trials tab.

Check for U.S. clinical trials from NANT or NCI's list of cancer clinical trials that are now accepting patients with neuroblastoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from NANT http://www.nant.org or NCI Web site. http://www.cancer.gov/clinicaltrials


To Learn More about Neuroblastoma

For more information about Neuroblastoma, see the following:


Get More Information from the National Cancer Institute (NCI) 


Call:
 1-800-4-CANCER

For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 5:00 a.m. to 5:00 p.m., Pacific Time. A trained Cancer Information Specialist is available to answer your questions.

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