Neuroblastoma is a rare disease in which a solid
tumor (a lump or mass caused by uncontrolled or abnormal cell growth)
is formed by special nerve cells called neuroblasts. Normally, these
immature cells grow and mature into functioning nerve cells. But in
neuroblastoma, they become cancer cells instead.
Neuroblastoma most commonly starts in the tissue
of the adrenal glands, the triangular glands on top of the kidneys
that produce hormones responsible for controlling heart rate, blood
pressure, and other important functions. Like other cancers,
neuroblastoma can spread (metastasize) to other parts of the body,
such as the lymph nodes, skin, liver, and bones.
It occurs almost exclusively in infants and
children and is slightly more common in boys than in girls. Children
diagnosed with neuroblastoma are usually younger than 5 years old,
with the majority of new cases occurring among those younger than 1
year old.
Signs and Symptoms
The effects of neuroblastoma can vary widely
depending on where the disease first started and how much it has
spread to other parts of the body. The first symptoms are often vague
and may include irritability, fatigue, loss of appetite, and fever.
But because these early warning signs can develop gradually and mimic
those of other common childhood illnesses, neuroblastoma can be
difficult to diagnose.
In young children, neuroblastoma often is
discovered when a parent or doctor feels an unusual lump or mass
somewhere in the child's body — most often in the abdomen, though
tumors also can appear in the neck, chest, and elsewhere.
The most common signs of neuroblastoma are caused
by the tumor pressing on nearby tissues as it grows or by the cancer
spreading to other areas. These signs vary depending on how much the
cancer has grown and where it has spread.
For example, a child may have:
- a swollen stomach, abdominal pain, and decreased appetite (if the tumor is in the abdomen)
- bone pain or soreness, black eyes, bruises, and pale skin (if the cancer has spread to the bones)
- weakness, numbness, inability to move a body part, or difficulty walking (if the cancer presses on the spinal cord)
- drooping eyelid, unequal pupils, sweating, and red skin, which are signs of nerve damage in the neck known as Horner's syndrome (if the tumor is in the neck)
- difficulty breathing (if the cancer is in the chest)
Treatment
Most cases of neuroblastoma require treatment. The
type will depend on several factors including the child's age, the
characteristics of the tumor, and whether the cancer has spread.
Children with low-risk or intermediate-risk
neuroblastoma have a good chance of being cured. However, about
one-half of all children with neuroblastoma have the high-risk type,
which can be difficult to cure.
Typical treatments for neuroblastoma include
surgery to remove the tumor, radiation therapy, and chemotherapy. If
the tumor hasn't spread to other parts of the body, surgery is
usually enough.
Unfortunately, in most cases the neuroblastoma has
spread by the time it's diagnosed. In these cases, chemotherapy and
surgery are the primary treatments and may be performed in
conjunction with radiation therapy and stem cell or bone marrow
transplantation.
Newer treatment options include tumor vaccines and
immunotherapy using monoclonal antibodies, special substances that
can be injected into the body to seek out and attach to cancer cells.
They're sometimes used to deliver drugs or other treatments directly
to cancer cells, helping to improve treatment.
Prognosis
With treatment, many children with neuroblastoma
have a good chance of surviving. In general, neuroblastoma has a more
favorable outcome if the cancer hasn't spread or if the child is
younger than 1 year old when it's diagnosed. High-risk neuroblastoma
is harder to cure and is more likely to become resistant to standard
therapies or come back (recur) after initially successful treatment.
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