Neuroblastoma

James Whiting

Statistics: 2012
external image neuroblastoma1.jpg
external image neuroblastoma1.jpg

  • 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.

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.

Sources

http://www.cancer.org/Cancer/Neuroblastoma/DetailedGuide/neuroblastoma-key-statistics
http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002381/
http://www.ncbi.nlm.nih.gov/pubmed/8308685