I've finally started to read the book on Childhood Cancers that the social worker at Stanford recommended. There was a chapter on neuroblastoma that I found quite interesting and I wanted to relay some of that information here. The following text comes from the book, "Childhood Cancer - A Parent's Guide to Solid Tumor Cancers".
Introduction
Neuroblastoma is a cancer of the sympathetic nervous system. The average age at diagnosis is two years, and the majority of children are diagnosed before the age of five years. While neuroblastoma can arise at any place along the sympathetic nervous system from the neck to the pelvis, more than half of these cancers originate in the adrenal gland in the abdomen. Symptoms of this disease vary depending on the location of the primary tumor.
The sympathetic nervous system
The human body is equipped with an impressive information system. The central nervous system (CNS) includes the brain and the spinal cord. The brain acts as the central control room, while the spinal cord operates as a link to other important elements of the nervous system. The peripheral nervous system (PNS) connects the CNS to other organs and systems. The network of nerves that make up the sympathetic nervous system is part of the PNS. Chemical messengers, called neurotransmitters, relay information throughout the body using these two systems.
The sympathetic nervous system performs automatically in response to the environment and emotions. For example, if a person is surprised or angered, the sympathetic nervous system leaps into action by accelerating the heartbeat, increasing blood sugar, and cooling the body through perspiration.
The adrenal glands are small glands that sit on top of each kidney, and are responsible for producing many different hormones. The adrenal glands has two parts. The outer portion, called the cortex, secrets hormones such as cortisone and aldosterone that are used by the body for fluid and electrolyte balance. The central portion, called the medulla, produces hormones that help the body respond to stress.
Who gets neuroblastoma?
Neuroblastoma accounts for approximately 8 to 10 percent of all childhood cancers. Each year more than 600 new cases are diagnosed in the United States and an additional 65 are diagnosed in Canada. Boys are diagnosed more often that girls, and there is a slight higher incidence among white children than among black children. About half of all neuroblastomas are diagnosed by 2 years of age and about 75 percent are diagnosed before 5 years of age. Neuroblastoma is the most common cancer diagnosed in the first year of life.
Genetic factors
Neuroblastoma arise spontaneously (no known cause) in the vast majority of children diagnosed with the disease. Children with some disorders, however, have a higher risk of developing neuroblastoma. Two examples of these associated disorders are Hirschsprung disease (a developmental abnormality that involves the colon) and von Recklinghausen neurofibromatosis (NF 1).
Approximately 1 to 2 percent of children diagnosed have a family history of neuroblastoma. Affected children from these families are usually diagnosed as infants and have multiple primary tumors. Children with familial neuroblastoma can have tumors that develop in various ways, however. Some have tumors that spontaneously disappear, while others have a very aggressive form of the disease. Chromosome 16 is the likely location of the gene that predisposes people to hereditary neuroblastoma.
Environmental factors
Although certain types of cancer have been linked to environmental factors, this is not true for neuroblastoma. Many research studies have been conducted to evaluate possible environmental causes, but no conclusive answers have yet been found.
Staging
I'm only going to include the stage that was diagnosed for Jonathan.
Once a diagnosis of neuroblastoma has been made, the oncologist will order further tests and scans to determine the extent (stage) of the disease. The International Neuroblastoma Staging System (INSS) was developed to provide researchers with a system to compare data and facilitate the international exchange of information.
Using the INSS, neuroblastoma is categorized into five distinct stages:
Stage 4: The tumor has spread beyond the region of the main tumor, to distant lymph nodes, bone, bone marrow, liver, and / or other organs (except as defined for stage 4S).
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