Neuroblastoma is a
arises in immature nerve cells
of the sympathetic nervous system called neuroblasts. It is developed mostly in young children. It is a cancer of embryonic stem cells of the nerve tissue, and usually begins in the adrenal glands, neck, abdomen, chest or spinal
cord. Neuroblastoma occurs when neuroblasts undergo changes
that make their growth becomes abnormal,
leading to development of cancerous or malignant tissue. The tumor can be associated
with certain genetic disorders such as Hirschsprung's disease (please see
The nervous system consists of the central nervous system (CNS) and peripheral
nervous system (PNS). CNS comprises the brain and the spinal cord; and the
PNS includes parts of the nervous system that are located outside of the CNS.
The nervous system consists of the somatic
nervous system, which controls the voluntary body movements
such as walking; and the autonomic nervous system that controls the involuntary body functions such as breathing and
The autonomic nervous system includes the
sympathetic nervous system and the parasympathetic
nervous system. It runs
various activities of the body to a state
of rest while the sympathetic nervous system prepares the body for action.
The nerves of this system control
flight response. They react to situations that
require strength and perception, mainly situations that cause fear, anger, excitement
Epidemiology and Evolution
The main age of Neuroblastoma diagnosis in
children is between one and two years. Its incidence is approximately one case per
100,000 children. This remains the
most common cancer in children under one
year. It is mainly a childhood cancer, although a few cases have
been reported in adults.
Neuroblastoma is known to be one of the
few cancers to present regressions of an undifferentiated state to a
completely non-cancerous (benin) cellular
state, which can lead to spontaneous healing.
This favorable trend is, however, far
from constant. In fact, evolution is highly variable.
and risk Neuroblastoma
Neuroblastoma causes are really known to scientists. There is no
known single cause of neuroblastoma. Most cancers are due to many risk
factors. However, some children with
neuroblastoma have no identifiable risk
Age and gender seem to play certain role in its development. Approximately 80%
of neuroblastoma cases occur before the age of 5
years. This is the most common cancer in infants less than 1
year. The disease is rare in children over 10 years. It
affects slightly more boys
than girls: for 5
boys who develop neuroblastoma, 4
girls have it.
Scientists believe that the following factors increase
the risk of developing neuroblastoma
Family History - Approximately 1-2% of children who are diagnosed
with neuroblastoma have a family
history of the disease, especially if the cancer
has manifested in one of
their siblings or with their identical twin.
Genetic Disorders - It
is known that neuroblastoma tends to develop in children with genetic
disorders relating to conditions that affect
neural crest cells. These conditions include:
DiGeorge syndrome, also called 22q11.2 deletion
hypoglycemia and pancreatic
Von Recklinghausen's disease(VR), a rare type of medical
condition also called neurofibromatosis type 1
Other Possible risk factors
The fact that most cases of neuroblastoma occur at a very young
age, It is also believed that prenatal exposure to alcohol
due to alcohol consumption of the mother during pregnancy
may interfere with normal development of
neural crest cells, thus causing the disease. Certain drugs such as diuretics,
anticonvulsants, oral contraceptives and fertility hormones are
Signs and Symptoms of
symptoms vary depending
on the organ affected. The disease can develop anywhere
along the peripheral nervous system,
then its signs and symptoms are not easily recognizable at the beginning.
However, the most common symptoms occur because the tumor presses on nearby tissues
as it develops, or when the cancer has spread to the bones. In most cases, the neuroblastoma does not cause malfunction of the peripheral nervous system.
Signs and symptoms of neuroblastoma may also
be caused by other medical conditions. It is important to consult a physician if of these
any unusual symptoms occur.
in the Abdomen
in the Chest
in the Neck area
in the spinal cord
swelling of the
breathing or shortness of breath
and irritability due to increased
production of catecholamines
in the iris of the
inability to sweat
abnormalities (wobbly gait)
or difficulty in urination
paleness, easy bruising and bleeding
Depending on the severity of the disease there may be other signs
and symptoms, which may include:
increase in blood pressure
the face and neck
eyes - dark circles and protruding eyes
bluish masses under the skin
Opsomyoclonus(dancing eyes dancing feet
severe diarrhea caused by an
increased production of a hormone called vasoactive
intestinal peptide (VIP)
Neuroblastoma treatment depends on the stage
and location of the tumor. When
cancer is located, it is usually
curable. However, the life expectancy of
children with advanced neuroblastoma
despite aggressive therapies.
advanced cases, platinum-compound
administered, along with Etoposide alternated with vincristine, doxorubicin and cyclophosphamide.
Topotecan appears to be given in serious
cases, but relapses are frequent. Bone narrow transplant after aplastic chemotherapy
is often include in the treatment options.
Some oncologists use surgery, radiation therapy or other chemotherapy drugs.
therapy and other forms of therapy are sometimes recommended. Usually, the health care giver discusses the options with the parents of the
sick child to determine the best treatment plan
to be adopted.
is designed to
meet the unique needs of each child with
Neuroblastoma; there is no unique regimen. The final decision is based
involved and category of the tumor being treated. Children with neuroblastoma are classified as high, medium or low, depending on the age of the child, stage of cancer or characteristics of neuroblastoma cells.
Trials - Clinical trials are designed to find
better methods of prevention, detection and treatment of neuroblastoma. In USA,
few clinical trials are available to
children with neuroblastoma.