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Neuroblastoma

By

Kee Kiat Yeo

, MD, Harvard Medical School

Reviewed/Revised May 2023
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Neuroblastoma is a common childhood cancer that grows in parts of the nervous system or adrenal glands.

  • What causes neuroblastoma is often not known.

  • Symptoms depend on where neuroblastomas develop, such as the abdomen, chest, bone, skin, or spinal cord.

  • Diagnosis usually involves an imaging test and a biopsy.

  • Treatment depends on the child's age and the specific characteristics of the cancer and may include surgery, chemotherapy, and radiation.

A neuroblastoma develops in a certain kind of nerve tissue located in many places of the body. It usually originates in nerves in the abdomen or chest, most commonly in the adrenal glands Overview of the Adrenal Glands The body has 2 adrenal glands, one near the top of each kidney. They are endocrine glands, which secrete hormones into the bloodstream. Each adrenal gland has 2 parts. Medulla: The inner part... read more (located above each kidney). In over half of the children, the cancer has spread to other parts of the body by the time a doctor is consulted.

Neuroblastoma is the most common cancer among infants. About 90% of all neuroblastomas occur in children younger than 5 years. With very rare exceptions, neuroblastomas occur only in children.

The cause of neuroblastoma is often not known. Most of these tumors occur spontaneously. Rarely, neuroblastomas run in families.

Did You Know...

  • Neuroblastoma is the most common cancer among infants.

Symptoms of Neuroblastoma

The symptoms of neuroblastoma depend on where the neuroblastoma originated and whether and where it has spread, as in the following:

  • Originating in the abdomen: The most common symptoms include a large abdomen, a sensation of fullness, decreased appetite, 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. 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.

  • 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 Horner Syndrome Horner syndrome affects one side of the face, causing the eyelid to droop, the pupil to become small (constricted), and sweating to decrease. The cause is disruption of the nerve fibers that... read more 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 one side of the face.

Most neuroblastomas produce catecholamines. Catecholamines are hormones that tend to increase the heart rate and cause anxiety. Other syndromes associated with cancer (called paraneoplastic syndromes Paraneoplastic Syndromes Paraneoplastic (associated with cancer—see also Overview of Cancer) syndromes occur when a cancer causes unusual symptoms due to substances that circulate in the bloodstream. These substances... read more ), such as uncontrollable eye movements (opsoclonus) and quick contractions of the arms and legs (myoclonus), watery diarrhea, or high blood pressure, can occur.

Diagnosis of Neuroblastoma

  • Computed tomography (CT) or magnetic resonance imaging (MRI)

  • Biopsy

  • Sometimes bone marrow analysis and urine tests

Early diagnosis of a neuroblastoma is not straightforward.

After birth, if the cancer has grown large enough, a doctor may be able to feel a lump in a child's abdomen.

A urine sample can be tested for excessive production of catecholamine (which can be produced by the tumor).

To see whether the cancer has spread, the doctor may do the following:

  • CT or MRI of the abdomen, pelvis, and chest, and sometimes the brain

  • Biopsy of any identified mass

  • Examination of a sample of bone marrow

  • A bone scan or a scan that uses a radioactive material known as metaiodobenzylguanidine (MIBG) that helps doctors see whether the neuroblastoma has spread

The doctor uses 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

  • Sometimes stem cell transplantation

  • Immunotherapy

Treatment of neuroblastoma is based on the risk category.

Children who have intermediate-risk or high-risk disease are given chemotherapy drugs such as vincristine, cyclophosphamide, doxorubicin, etoposide, and cisplatin.

Prognosis for Neuroblastoma

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 features of the DNA within the tumor cells). Younger children whose cancer has not spread have the best prognosis.

The survival rates for children who have low-risk and intermediate-risk disease are about 90 to 95%.

The survival rate for children who have high-risk disease was about 15% but has improved to greater than 50% with newer and intensified methods of combined therapy.

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: If Your Child Is Diagnosed With Cancer: A resource for parents and loved ones of a child who has cancer that provides information about how to cope with some of the problems and questions that come up just after a child is diagnosed

Drugs Mentioned In This Article

Generic Name Select Brand Names
Oncovin, Vincasar PFS
Cyclophosphamide, Cytoxan, Neosar
Adriamycin, Adriamycin PFS, Adriamycin RDF, Rubex
Etopophos, Toposar, VePesid
Platinol, Platinol -AQ
A Mulsin, Aquasol A, Dofsol-A
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