Neuroblastoma

By: Autumn Dow

Neuroblastoma

What is it?

Neuroblastoma is, simply put, a cancer where malignant cells form in nerve tissue. It most often begins in the adrenal glands, which are on top of the kidneys, but can also form in nerve tissue in the neck, chest, abdomen, or spine. Neuroblastoma metastasizes rapidly to places like the cortical bone, bone marrow, and lymph nodes (common). Though less common, it can also metastasize to the lungs and brain, as well as the skin.

Signs, Symptoms, and Detection

  • Neuroblastoma is most commonly found in children under 5 years of age
  • It is usually found when the tumor begins to grow quite large
  • Tumors in the abdomen or pelvis- the child might not want to eat (leads to weight loss)
  • Tumors can also press against or grow into blood and lymph vessels in the abdomen or pelvis, which can stop fluids from getting back to the heart.
  • Tumors in the chest could press on the superior vena cava, which could lead to swelling in the face, neck, arms, and upper chest (sometimes with a bluish-red skin color)
  • Tumors could also press on the throat or windpipe, which can cause coughing, trouble breathing, and trouble swallowing
  • Pressure on other nerves near the spine could affect the child's ability to feel or use their arms or legs
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Treatment

Families will generally find a cancer center for children with a team of highly specialized doctors, including a:


  • Pediatric oncologist (chemotherapy and other medicines)
  • Pediatric radiation oncologist (radiation therapy)
  • Pediatric cancer surgeon (surgery- if situation is critical)

Prognosis

Prognosis depends on a wide range of factors which include:


  • Size and location of the tumor(s)
  • Response of the tumor to therapy
  • Child's tolerance of specific medications, procedures, and therapies



Low-risk patients carry more than a 90% long-term survival rate, but patients with much more advanced stages of neuroblastoma have a much less optimistic prognosis. Patients under the age of 18 months have a greater chance of survival than those who are older.

For significant cases, the survival rate after diagnosis is about 5 years.

Risk Factors

  • Mutations in the ALK and PHOX2B genes have been shown to increase the risk of developing sporadic and familial neuroblastoma.
  • Deletion of certain regions of chromosome 1 and chromosome 11 are also associated with neuroblastoma.
  • Only about 1-2% of affected individuals have familial neuroblastoma. The other percent is affected by sporadic neuroblastoma.

Research

Research on neuroblastoma is under way in university hospitals, medical centers, and other insititutions. With these studies, information is used to learn about better treatment options, and because of this, survival rates have increased. Also, scientists are beginning to work with immunotherapy, which is therapy using cancer vaccines.
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