Pathophysiology of Neuroblastoma

Elaboration, 2018
7 Pages, Grade: 1


Neuroblastoma refers to a malignant cancer disease that affects the nerve tissue in
different parts of the body, primarily the nerve tissue of the spinal cord, adrenal glands, chest
and neck. However, the disease is believed to begin from the adrenal glands and spread to the
other nerve tissues. In most cases, neuroblastoma sets in the early childhood, especially in
children below the age of 5 years, and persists into adulthood. In rare occasions, malignant
cancer cells form during the fetal development stages of the fetus, long before the child is
born, as it has been identified in ultrasound results. In practice, neuroblastoma presents some
difficulties in both management and diagnosis because; this cancer spreads rapidly and this
explains why diagnosis is usually done when the disease has already metastasized to the
bones, lymph nodes, skin and the liver
Currently, neuroblastoma is ranked third among the most common cancers in children
after cancer of the central nervous system and leukemia. In Ireland, the prevalence rate of
neuroblastoma has been identified as 5.6%, which is relatively higher that the prevalence
rates of the entire Europe that is estimated to be 3.4%
. Research data on the prevalence of
childhood cancers in the United Kingdom indicates that, neuroblastoma has the highest
incident rate in children aged three years in which its rate is estimated to be 22% per million
. Globally, neuroblastoma accounts for 7% of childhood cancers
Neuroblastoma is usually manifested through numerous signs and symptoms.
Ordinarily, the most common signs and symptoms are associated to the pressure exerted by
the tumor on the adjacent tissues. Some of the most common signs and symptoms of this
disease include the formation of a lump in the neck, chest or the abdomen. In some occasions,
the disease is manifested by swollen stomach, especially in infants in which breathing
complications develop. Paralysis is also another significant sign of neuroblastoma in which
infants lose the ability to move some body parts such as the head, legs and arms. It can also
be manifested by dark circles that form around the infant's eyes. Other principal symptoms
include bleeding characterized with petechiae, severe watery diarrhea, high blood pressure
and jerky muscle movements. However, it is worth noting that, pains in the bones serve as
one of the most principal symptoms of neuroblastoma because it affects the bones and bone
marrow during its initial stages
Currently, there is no known cause of neuroblastoma, just like it is the case with most
cancers. However, some risk factors have been identified. Research indicates that age and
family history are some of the most principal risk factors that are associated with
neuroblastoma. In most cases, this cancer occurs in populations with less than 10 years in
which infants and babies are the most affected. This is so because its incidence rates have

been found to be highest among children below the ages of 3 years, and it is relatively low in
children aged 10 years and above. Therefore, age appears to be one of the most significant
risk factors for neuroblastoma. The second risk factor for neuroblastoma is the family history
of inherited genetic abnormalities
. Research indicates that, 1-2% of children who suffer
from neuroblastoma inherited the genetic abnormality from their relatives. As such, family
history of neuroblastoma is considered as one of the principal risk factors of the disease.
However, it is worth noting that, most neuroblastomas do not seem to exhibit inheritance
On the other hand, diagnosis of neuroblastoma encompasses numerous tests and
investigations ranging from blood tests, biopsy and bone marrow tests to scans. Ideally, tests
are usually done to ascertain whether a child has neuroblastoma or not. They also help in
determining the spread of the cancer because neuroblastomas exhibit rapid metastasis. On the
other hand, nuclear medicine scans are done to identify the exact location of the tumor, in
order to decide on the most appropriate treatment option. Some of the most reliable tests for
diagnosing neuroblastoma are Vanillylmandelic acid (VMA) test and Homovanillic acid
(HVA) test, in which urine samples are collected and analyzed for the presence of
homovanillic acid and vanillylmandelic acid levels. These tests are essential in the diagnosis
of neuroblastoma because; 9 of 10 children with neuroblastoma show increased levels of
these two metabolites in their urine; thus, it helps in confirming diagnosis. In addition, VMA
and HMA tests play a significant role in monitoring the progress of treatment by observing
the decrease of these compounds in urine samples of neuroblastoma patients. Other reliable
diagnostic methods are biopsies and metaiodobenzyl guanine (MIBG) scans. In biopsy
diagnosis, tissue lesions are collected during an operation for microscopic and histological
investigations. On the other hand, MIBG scans employ the principle of radiation scanning to
determine the exact location of neuroblastoma tumors. In this diagnostic approach,
metaiodobenzyl guanine, a radioactive substance is injected into the patient's blood
circulation, whereby it is taken up by tumor cells; thus allowing easy location. Therefore,
radiation scanning determines the levels of radioactive iodine to identify the location of the
Treatment for neuroblastoma involves numerous treatment modalities. These
treatment options usually depend on the size and position of the tumor, as well as, the age of
the child. They also depend on the tumor biology, primarily regarding the extent of
metastasis. Over the years, chemotherapy, surgery and radiotherapy have been the mainstay
of neuroblastoma treatment. However, new therapies including stem cell therapy and

monoclonal antibody treatment are currently being incorporated in the treatment of
In practice, surgery is usually used as the most appropriate treatment option for
localized tumors. This approach involves the removal of the tumor to prevent neuroblastoma
from spreading to other parts of the body. In most cases, localized tumors are observed
during the early stages of neuroblastoma. During the early stages of the cancerous tumor
development, lymph nodes and the bones are not affected so, an operation to remove the
tumor prevents further development of malignant tumor cells. Evidence shows that surgery in
combination with chemotherapy results into cure of neuroblastoma when diagnosis is done
during its onset. However, it is worth noting that, some of the localized tumors are difficult to
remove owing to their locations such as within the bone tissue or the spinal cord.
In high-risk neuroblastoma, radiotherapy is used as the most appropriate treatment
option. Radiotherapy is also useful in treating metastasized tumors in which high-energy
radiation rays are used to destroy the cancerous cells. However, it is worth noting that there
are two forms of radiotherapy: external and internal radiotherapy. In external radiotherapy,
high-energy rays from an external machine are directed to the affected regions; thus, causing
destruction to the tumor cells while preserving normal cells. On the other hand, internal
radiotherapy involves the use of radioactive MIBG, in which high doses of radioactivity kills
the cancer cells
On the other hand, chemotherapy is used in metastasized, as well as, in high-risk
tumors. This therapy aims at killing cancer cells in the body through the use of cytotoxic
(anti-cancer) drugs. Evidence shows that, cytotoxic drugs shrink tumors; thus, preventing the
spread of neuroblastoma in the body. However, it is worth noting that, intensive
chemotherapy is required in achieving appreciable treatment outcomes.
Moreover, new therapies have been developed including the stem cell coupled high-
dose chemotherapy and monoclonal antibody treatment. In treating metastasized
neuroblastoma, stem cell support is coupled with high-dose chemotherapy, primarily after the
initial stages of chemotherapy treatment
. Ordinarily, high-dose chemotherapy destroys
cancer cells, although it causes harm to the normal cells because high levels of cytotoxic
drugs are lethal to both cancerous and healthy cells, especially the bone marrow cells. This
implies that, the synthesis of blood cells is impaired by the use of high-dose chemotherapy
Therefore, stem cells are given in the form of a drip to a child after high-dose chemotherapy
to restore proper hematopoietic processes of the bone marrow leading to the replacement of
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Pathophysiology of Neuroblastoma
Egerton University
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Neuroblastma, cancer
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Patrick Kimuyu (Author), 2018, Pathophysiology of Neuroblastoma, Munich, GRIN Verlag,


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