Handbook of Genetic Counseling/Ewing's Sarcoma

Ewing's Sarcoma

What is Ewing's sarcoma/PNET?

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  • a cancer that can start in bone or soft tissues
  • most common sites for are the pelvis, the thigh, and the trunk of the body
  • peak ages are 10-20, but younger children and older adults can also get it
  • half are under 15 years
  • less common before 5 or after 30
  • no known cause
  • cells in the tumours have a piece of chromosome 11 moved to chromosome 22
  • 85% contain chromosomal translocation at the t(11;22)(q24;q12)
  • the genetic change occurs only in the tumour cells, not in the sperm or egg
  • 2.5% of cases of childhood cancer
  • development of the tumour appears to be in some way related to periods in life with rapid growth, hence the average for tumour development is 14-15 years
  • slightly more common in boys than in girls
  • most common early signs are pain and swelling
  • can spread to other parts of the body
  • always requires treatment to the whole body (chemotherapy, surgery, radiation therapy)

Symptoms

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  • vary from person to person depending on the location and size of the cancer
  • most common symptoms are pain and swelling or tenderness in the affected area
  • pain may become very intense when the tumour is located near important nerves, like in the sacrum, pelvis or spine
  • swelling is often seen, especially when the log bones of the arms or legs are affected
  • tiredness, fever, weight loss, and anaemia

Notes

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The information in this outline was last updated in 2002.