Ewing's Sarcoma



What is Ewing's family of tumors?

Cancers in the Ewing's family of tumors are made of primitive cells, which are cells that haven't yet decided what type of cell they are.  They look blue due to the staining so the cells are referred to as small round blue cells. Picture.

The Ewing's family of tumors include: 

bullet Ewing's tumor of bone
bulletExtraosseus Ewing's (tumor growing outside of the bone)
bulletPrimitive neuroectodermal tumor (PNET), also known as peripheral neuroepithelioma
bullet Askin's tumor (PNET of the chest wall).  

All of these are frequently referred to Ewing's sarcoma in the popular and medical literature. These are a subtype of sarcoma, which are tumors in the connective tissues.

Since 90% of Ewing's sarcoma tumors occur in bones, it is commonly thought of as a bone cancer. 

How is it treated?

Currently, the treatments of both soft tissue and bone tumors are the same. A number of clinical trials have been performed on children, so the treatment is quite standardized.  Assuming the cancer responds to chemotherapy, the standard treatment is:

bullet14-17 cycles of chemotherapy, alternating between 2 regimens of drugs
bulletResection surgery if possible.  This will frequently involve limb-sparing surgery and donor bones if there is bone involvement.
bulletDaily radiation treatments for 6 weeks to the primary site

Ewing's sarcoma is an aggressive cancer, and requires 9 months to a year to treat in the best case. If the cancer doesn't respond to "first line" chemo, and if there is known disease, there are others to try.  If those don't work, as for most types of cancer, there are clinical trials to try.

What are the typical treatment scans?

If Ewing's sarcoma spreads, it normally spreads to the lungs, bones and bone marrow. So, chest CT scans are performed to check the lungs.  If the primary tumor was in the bone, bone marrow biopsies are performed.  Bone scans check for tumors in the bones.

What is the prognosis?

An important characterization is whether the cancer is local, or has spread (i.e., has metastized). If there are no metastases at diagnosis, the 5-year prognosis is 70% for children and 50% for adults.  If there are metastases, the prognosis is much lower (10-25%).  And, if metastases develop while in treatment, the prognosis is very grim.

Who develops Ewing's sarcomas?

Ewing's sarcoma is considered a pediatric cancer as it has a higher incidence in children than in adults.  The median age is 13 years old.  There are approximately new 250 cases in children in the US per year, and approximately 25 in adults. 


Copyright 2003 The Shriver Family: Last modified: 01/06/04.