Handbook of Genetic Counseling/Ewing's Sarcoma

Ewing's Sarcoma

What is Ewing's sarcoma/PNET?

 * 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

 * 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