Neuroblastomas are cancers that grow in the adrenal glands or other parts of the nervous system, including the chest, neck, and abdomen. These tumors develop during childhood. All neuroblastomas are cancerous (malignant).
Symptoms depend on where the tumors develop.
Diagnosis usually involves an imaging test and a biopsy.
Treatment depends on the child's age and the specific characteristics of the tumor and may include a combination of surgery, chemotherapy, radiation therapy, immunotherapy, and other medications.
(See also Overview of Childhood Cancer.)
Neuroblastoma is the most common cancer among infants. About 85% of all neuroblastomas occur in children younger than 5 years of age, and about 95% occur in children younger than 10 years of age. Very rarely, neuroblastomas occur in adults.
A neuroblastoma develops in a certain kind of nerve tissue located in many places of the body. The tumor usually originates in nerves in the abdomen or chest, most commonly in the adrenal glands, which are located above each kidney. In over half of children, the cancer has spread to other parts of the body (metastasized) by the time a doctor is consulted.
The cause of neuroblastoma is often not known. Most of these tumors occur spontaneously. Rarely, neuroblastomas run in families.
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Symptoms of Neuroblastoma
The symptoms of neuroblastoma depend on where the tumor originated and whether and where it has spread:
Originating in the abdomen: The most common symptoms include a large abdomen, a sensation of fullness, decreased appetite, discomfort, irritability, and abdominal pain.
Originating in the chest or neck: The child may cough or have difficulty breathing.
Spread to the bones: The child has bone pain. If the cancer reaches the bone marrow, the number of various types of blood cells may be reduced, causing certain other symptoms. For example, a reduced number of red blood cells (anemia) causes fatigue and sometimes pale skin (pallor). A reduced number of platelets causes easy bruising and tiny purple spots on the skin. A reduced number of white blood cells increases the risk of infections.
Spread to the skin: Lumps appear, which are sometimes blue- or purple-colored.
Spread to the spinal cord: The arms and legs may feel weak and numb, or children may not be able to voluntarily control some body parts.
Less commonly, children have symptoms of a disorder called Horner syndrome. In Horner syndrome, a tumor in the neck presses on nerves that affect one side of the face. Children have a drooping eyelid, a small pupil, and decreased sweating on the affected side of the face.
Most neuroblastomas produce catecholamines. Catecholamines are hormones that tend to increase the heart rate and cause anxiety. Neuroblastomas can also cause symptoms that are not directly caused by the tumor itself called paraneoplastic syndromes. Symptoms of these syndromes include uncontrollable eye movements (opsoclonus), quick contractions of the arms and legs (myoclonus), watery diarrhea, and high blood pressure.
Diagnosis of Neuroblastoma
Computed tomography (CT) or magnetic resonance imaging (MRI)
Biopsy
Blood and sometimes urine tests
Sometimes bone marrow analysis
Before birth, routine prenatal ultrasound studies occasionally detect neuroblastoma in a fetus.
After birth, neuroblastoma is most commonly found when children are evaluated for symptoms they are having. If the cancer has grown large enough in the abdomen, a doctor may be able to feel a lump.
A doctor who suspects a neuroblastoma does a CT or MRI scan of the abdomen. If a tumor is found, a sample is taken and sent to a laboratory for examination (biopsy) and an analysis of the tumor's genes.
Doctors do blood tests and may test urine for excessive levels of catecholamines produced by the tumor.
Less often, doctors remove a sample of bone marrow to look for cancer cells (see Bone Marrow Examination).
To see whether the cancer has spread, doctors do the following:
CT or MRI scan of the abdomen, pelvis, and chest, and sometimes the brain
Examination of a sample of bone marrow
A scan that uses a radioactive material known as metaiodobenzylguanidine (MIBG scan), a bone scan, or a special type of positron emission tomography (PET) scan
The doctors use all of this information to determine whether the tumor is low risk, intermediate risk, or high risk.
Treatment of Neuroblastoma
Surgical removal
Chemotherapy
Sometimes radiation therapy or treatment with a radioactive material called metaiodobenzylguanidine (MIBG)
Sometimes stem cell transplantation and high doses of chemotherapy
Immunotherapy, isotretinoin, granulocyte-macrophage colony stimulating factor, and sometimes eflornithine
(See also Cancer Treatment Principles.)
Treatment of neuroblastoma is based on the risk category.
Neuroblastoma that has not spread and is low risk can often be removed and cured by surgery.
Children who have intermediate-risk or high-risk neuroblastoma are given a chemotherapy medication such as vincristine, cyclophosphamide, doxorubicin, etoposide, or cisplatin.
High-dose chemotherapy with stem cell rescue (sometimes referred to as a type of stem cell transplant) is frequently used for most children with high-risk neuroblastoma. To reduce the risk of the cancer coming back after high-dose chemotherapy and stem cell transplantation, children are given a combination of immunotherapy (to stimulate their body's immune system against the cancer), the medication isotretinoin, and a substance that stimulates the body's production of white blood cells (granulocyte-macrophage colony stimulating factor). Some children are given an additional medication called eflornithine.
Radiation therapy is used for children with high-risk neuroblastoma. It may be used for children with intermediate-risk neuroblastoma or for those whose tumors are inoperable. MIBG is another treatment option for these children. MIBG treatment uses a different, higher-energy form of radioactive iodine than the MIBG scan used for diagnosis.
Immunotherapy in combination with chemotherapy is also used to treat children with neuroblastomas that do not respond to treatment or that come back after treatment.
Prognosis for Neuroblastoma
The prognosis depends on several factors such as the child's age at diagnosis, whether the tumor has spread, and certain characteristics of the tumor called biologic features (for example, how the tumor looks under a microscope and some genetic features within the tumor cells). Young children whose cancer has not spread have the best prognosis.
The survival rates for children who have low-risk or intermediate-risk neuroblastoma are about 95 to 97%.
The survival rate for children who have high-risk neuroblastoma is about 50 to 70%.
More Information
The following English-language resource may be useful. Please note that The Manual is not responsible for the content of this resource.
American Cancer Society: How to Cope if Your Child Has Cancer
Drug Information for the Topic
