What is Neuroblastoma

Mainly affecting children, neuroblastoma is a relatively rare cancer of the sympathetic nervous system -- a nerve network that carries messages from the brain throughout the body. Each year about 600 children in the United States will develop neuroblastoma. Generally developing in young children, it accounts for half of all malignancies in infants. These solid tumors, which take the form of a lump or mass, commonly begin in one of the adrenal glands, though they can also develop in nerve tissues in the neck, chest, abdomen, or pelvis. The adrenal glands, located above the kidneys, are specialized glands that release hormones which maintain blood pressure and respond to stress.

The cause of neuroblastoma is unknown, though most physicians believe that it is an accidental cell growth that occurs during normal development of the adrenal glands. Increased awareness and improved screening has contributed to a recent increase in the detection and incidence of neuroblastoma.

Symptoms

Neuroblastoma's first symptoms are often vague and may include fatigue and loss of appetite. Later on, symptoms depend on where the tumor takes root within the body. A tumor in the abdomen may cause a swollen belly and constipation. A tumor in the chest may cause breathing problems. Tumors pressing on the spinal cord cause a feeling of weakness. When this occurs, the child may be unable to walk. Because symptoms are so unclear, half of all neuroblastomas have already spread to other parts of the body by the time suspicions are raised and a diagnosis is made. 

Diagnosis

Since neuroblastoma's symptoms can be similar to symptoms of other, more common diseases and health complaints, there is often a delay in making the diagnosis. An oncologist or pathologist will be able to diagnose neuroblastoma once he or she reviews either a biopsy of the tumor or the results of urine and bone marrow tests.

Screening tests are available for neuroblastoma, but such tests will not find a neuroblastoma before it has spread throughout the body. For this reason, screening newborns or young children for neuroblastoma is generally not recommended.
Staging of Neuroblastoma

The international tumor staging system outlined below will be simplified in the very near future.

• Stage 1
  Localized tumor confined to the area of origin; complete gross resection, with or without microscopic residual disease; identifiable ipsilateral and contralateral lymph node negative for tumor.
• Stage 2A
  Unilateral tumor with incomplete gross resection; identifiable ipsilateral and contralateral lymph node negative for tumor.
• Stage 2B
  Unilateral tumor with complete or incomplete gross resection; with ispilateral lymph node positive for tumor; identifiable contralateral lymph node negative for tumor.
• Stage 3
  Tumor infiltrating across midline with or without regional lymph node involvement; or unilateral tumor with contralateral lymph node involvement; or midline tumor with bilateral lymph node involvement.
• Stage 4
  Dissemination of tumor to distant lymph nodes, bone marrow, bone, liver, or other organs except as defined by Stage 4S.
• Stage 4S
  Localized primary tumor as defined in Stage 1 or 2, with dissemination limited to liver, skin, or bone marrow (less than 10 percent of nucleated bone marrow cells are tumors).

Treatment 

Neuroblastomas are highly diverse in their behavior. Some will go away without any treatment, and others can be cured by surgery alone. But half of these tumors spread quickly to the bone and bone marrow and require one or more of the following treatments: chemotherapy, radiation therapy, stem cell transplantation, and immunotherapy. The right treatment for each child depends mainly on the child's age, the tumor's location, and where the tumor has spread to.

Surgery

Surgery is the oldest form of treatment for cancer. Most children with neuroblastoma will have surgery.

Chemotherapy

Chemotherapy is the use of chemicals or drugs to treat cancer. These drugs are swallowed or given through a vein. This type of treatment is systemic, which means that it reaches all areas of the body.

Immunotherapy

Immunotherapy works by getting the body's immune system (including white blood cells) to fight the cancer.

Radiation Therapy

Radiation therapy uses a large amount of x-rays (high-energy particles or waves) to kill cancer cells. Radiation therapy may be used to try to cure or control the disease. It can be used before surgery to shrink a tumor or after surgery to prevent the cancer from regrowing. In advanced cancer, radiation therapy may be used to treat symptoms that are caused by the cancer, such as pain.

Stem Cell or Bone Marrow Transplantation

Bone marrow is a fatty or spongy tissue found inside bones. The bone marrow in the hips, breastbone, skull, ribs, and spine contains stem cells. These bone marrow stem cells make blood, including red blood cells, white blood cells, and platelets. Under certain conditions, bone marrow stem cells circulate in the bloodstream -- and a special machine can collect the stem cells from the blood. Stem cell transplants are used for some patients whose bone marrow, because of disease or treatment, no longer produces enough blood cells. Most stem cell transplants for neuroblastoma use the patients' own (autologous) stem cells.