Neuroblastoma

Neuroblastoma often begins in the nerve tissue of the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. The adrenal glands make important hormones that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Neuroblastoma may also begin in the abdomen, in the chest, in nerve tissue near the spine in the neck, or in the spinal cord.

 

Neuroblastoma most often begins during early childhood, usually in children younger than 5 years of age. It is found when the tumor begins to grow and cause symptoms. Sometimes it forms before birth and is found during a fetal ultrasound.

By the time neuroblastoma is diagnosed, the cancer has usually metastasized (spread). Neuroblastoma spreads most often to the lymph nodes, bones, bone marrow, liver, and in infants, skin.

 

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • Age of the child at diagnosis.

  • Stage of the cancer.

  • Tumor histology (the shape, function, and structure of the tumor cells).

  • Whether there is cancer in the lymph nodes on the same side of the body as the cancer or whether there is cancer in the lymph nodes on the opposite side of the body.

  • How the tumor responds to treatment.

  • Whether there are certain changes in the chromosomes.

  • How much time passed between diagnosis and when the cancer recurred (for recurrent cancer).

    Prognosis and treatment options for neuroblastoma are also affected by tumor biology, which includes:

  • The patterns of the tumor cells.

  • How different the tumor cells are from normal cells.

  • How fast the tumor cells are growing.

  • Whether the tumor shows MYC-N gene amplification.

    The tumor biology is said to be favorable or unfavorable, depending on these factors. A favorable tumor biology means there is a better chance of recovery.

    In some infants, neuroblastoma may disappear without treatment. The infant is closely watched for symptoms of neuroblastoma. If symptoms occur, treatment may be needed.

     

    There are different types of treatment for patients with neuroblastoma.

    Different types of treatment are available for patients with neuroblastoma.

     

    Five types of standard treatment are used:

    Watchful waiting

    Watchful waiting is closely monitoring a patient's condition without giving any treatment until signs or symptoms appear or change.

    Surgery

    Surgery is used to treat neuroblastoma unless it has spread to other parts of the body. Depending on where the tumor is, as much of the tumor as is safely possible will be removed. If the tumor cannot be removed, a biopsy may be done instead.

    Radiation therapy

    Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

    The way the radiation therapy is given depends on the type and stage of the cancer being treated. External radiation therapy is used to treat neuroblastoma.

    Chemotherapy

    Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

    The use of two or more anticancer drugs is called combination chemotherapy. Some neuroblastomas become resistant to chemotherapy, reducing the benefit to the patient of their treatment.

    High-dose chemotherapy and radiation therapy with stem cell rescue

    High-dose chemotherapy and radiation therapy with stem cell rescue is a way of giving high doses of chemotherapy and radiation therapy and replacing blood -forming cells destroyed by cancer treatment for high-risk neuroblastoma. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient 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.

     

    What research is Garvan doing in this area?

    Dr Alex Swarbrick’s team is investigating the role for tiny RNA molecules, called microRNA, in the response of neuroblastomas to chemotherapy. In 2010 they showed that blocking a microRNA lead to dramatic killing of neuroblastoma cells in culture and in animal models of disease. Their recent work has found that microRNAs can regulate the sensitivity of neuroblastoma to chemotherapy, and by changing microRNA levels they can resensitise neuroblastomas to chemotherapy.

     

    This information is based on text taken from:

    National Cancer Institute: PDQ® Neuroblastoma Treatment. Bethesda, MD: National Cancer Institute. Date last modified <24/02/2014>. Available at: http://cancer.gov/cancertopics/pdq/treatment/neuroblastoma/Patient