Neuroblastoma

According to Children’s Oncology Group (COG), neuroblastoma is a solid tumor or cancer. It occurs in the developing cells of the sympathetic nervous system, called neuroblasts. The sympathetic nervous system is responsible for involuntary actions of the body, such as blushing, increasing heart rate, and dilating the pupils of the eye. The majority of tumors (65%) are located above the kidney. However, tumors can begin anywhere in the body. Other common sites are the chest, neck or pelvis. The disease often spreads from its "primary" location to the bone marrow, bones or lymph nodes. In fact, in many cases the disease has already spread at the time of diagnosis.

There are approximately 650 new cases of neuroblastoma diagnosed in the US annually. Neuroblastoma is the most common solid tumor outside of the brain in children. Most children are diagnosed as toddlers, but neuroblastoma can present in infants and older teenagers as well1.

 

Staging of Neuroblastoma

 

Stages of neuroblastoma include:

Stage 1: In stage 1, the tumor is in only one area and the entire tumor that can be seen is completely removed during surgery.

Stage 2: Stage 2 is divided into stage 2A and 2B.

  • Stage 2A: The tumor is in only one area and all of the tumor that can be seen cannot be completely removed during surgery.
  • Stage 2B: The tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery. Cancer cells are found in the lymph nodes near the tumor.

Stage 3: In stage 3, one of the following is true:

  • the tumor cannot be completely removed during surgery and has spread from one side of the body to the other side and may also have spread to nearby lymph nodes; or
  • the tumor is in only one area, on one side of the body, but has spread to lymph nodes on the other side of the body; or
  • the tumor is in the middle of the body and has spread to tissues or lymph nodes on both sides of the body, and the tumor cannot be removed by surgery.

Stage 4: Stage 4 is divided into stage 4 and stage 4S.

  • In stage 4, the tumor has spread to distant lymph nodes, the skin, or other parts of the body.
  • In stage 4S, the following are true:
  • the child is younger than 1 year; and
  • the cancer has spread to the skin, liver, and/or bone marrow; and
  • the tumor is in only one area and all of the tumor that can be seen may be completely removed during surgery; and/or
  • cancer cells may be found in the lymph nodes near the tumor.

 

Treatment of Neuroblastoma

 

For many types of cancer, stages are used to plan treatment. For neuroblastoma, treatment depends on risk groups. The stage of neuroblastoma is one factor used to determine risk group. Other factors are the age of the child, tumor histology, and tumor biology.

There are 3 risk groups: low risk, intermediate risk, and high risk.

  • Low-risk and intermediate-risk neuroblastoma have a good chance of being cured.
  • High-risk neuroblastoma may be difficult to cure.

Five types of standard treatment are used:

  • Surgery
  • Radiation therapy
  • Chemotherapy
  • Biologic therapy
  • Watchful waiting

 

New types of treatment are being tested in clinical trials.

 

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is one type of targeted therapy being studied in the treatment of neuroblastoma.

Monoclonal antibody therapy is a cancer treatment that uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to deliver drugs, toxins, or radioactive material directly to cancer cells.

High-dose chemotherapy and radiation therapy with stem cell transplant

High-dose chemotherapy and radiation therapy with stem cell transplant is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After chemotherapy and radiation therapy are completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

Other drug therapy

13-cis retinoic acid is a vitamin -like drug that slows the cancer's ability to make more cancer cells and changes how these cells look and act2.

Search for clinical trials in Georgia.

References for this page:

1Children’s Oncology Group (COG) at: http://www.curesearch.org/for_parents_and_families/newlydiagnosed/article.aspx?ArticleId=3242&StageID=1&TopicId=1&Level=1. (Accessed July 2016)

2Adapted from the National Cancer Institute's PDQ Database: http://www.cancer.gov/cancertopics/pdq/treatment/neuroblastoma/Patient/page1. (Accessed July 2016)