Neuroblastoma

= Neuroblastoma =

James Whiting
=== Statistics: 2012  ===
 * Neuroblastoma is the most common form of cancer in infants (under 1 year old)
 * Neuroblastoma accounts for 7% of all Childhood Cancers
 * There are 650 new cases in the U.S. every year. This number has not changed over the years.
 * In every 2 out of 3 cases the cancer has already metastasized when it is diagnosed.

What is Neuroblastoma?

 * Cancer that arises in immature nerve cells.
 * Affects mostly infants and children. It is rarely seen in anyone over the age of 10.
 * More common in boys than girls.
 * Caused by mutations in the ALK oncogene (common) or the PHOX2B gene (unusual).
 * Develops from tissues that form the sympathetic nervous system ( in charge of body functions such as heart rate, digestion, blood pressure and some hormone levels)
 * If the Neuroblastoma cells have extra copies of the MYCN oncogene, the cancer will be more dangerous and fast growing.
 * If the Neuroblastoma cells have overactive NTRK1 genes, the cancer is not as serious.
 * Neuroblastoma cells can send irregular hormone signals to organs in the body even if the cancer itself has not spread to them yet. These are called Paraneoplastic Syndromes.
 * Common developing points for tumors are the adrenal gland in the abdomen, next to the spinal cord, or in the chest.
 * Neuroblastoma can spread to bones (face, skull, pelvis, shoulders, arms, and legs), bone marrow, lymph nodes, liver, skin, and around the eyes (orbits).
 * Very rarely, a fetus will develop neuroblastoma in the womb, which can metastasize to the umbilical cord and lead to fetal death.
 * Only 1/2 of neuroblastoma tumors could pose a threat, others will just disappear or become benign over time.
 * Only 1/2 of neuroblastoma tumors could pose a threat, others will just disappear or become benign over time.

Symptoms

 * can be caused by the initial tumor, the spread of the tumor, and/or paraneoplastic syndromes.
 * Enlarged abdomen
 * Chronic cough or difficulty breathing
 * Bone pain or tenderness
 * Flushed red skin
 * Pale skin and bluish color around the eyes.
 * Profuse sweating
 * Rapid pulse
 * Fever
 * Constant diarrhea
 * Swelling
 * Blue lumps like blueberries indicate spreading.
 * All symptoms depend on the size and location of the tumor and its pressure on other organs.

Diagnosis

 * X-Ray
 * Biopsy
 * MRI
 * CT Scan
 * CBC Blood test
 * Hormone test
 * MIBG radioactive scan
 * Urine 24 Hour test for catechloamines, homovanillic acid (HVA), and vanillymandelic acid (VMA)

Treatment

 * Treatment depends on the risk level: low risk, intermediate risk, or high risk.
 * Surgery to remove tumor.
 * Chemotherapy
 * Radiation
 * Retinoid therapy: helps cancer cells differentiate into normal cells
 * High dose chemotherapy/radiation therapy with stem cell transplant a type of bone marrow transplant.
 * Immunotherapy (Biological Therapy)
 * In most cases more than one type of treatment is needed.
 * Some newer treatments involve monoclonal antibodies designed to target GD2 in the cancer cells and immune supporting drugs designed to bind to ALK in the cancer pathway.

Risk Reduction

 * There are no lifestyle choices that can either increase or reduce the risk of neuroblastoma.
 * Neuroblastoma is not hereditary but there are some (about 1 to 2% of all cases) that are familial and usually sporadically.
 * Some studies suggest that taking pre-natal vitamins or folic acid may reduce risks but this has not been officially verified.