Sam Butler Callahan Foundation | Ewing's Disease Q&A

Ewing Sarcoma Checklist

Ewing Sarcoma Checklist
Overview & Diagnosis
Treatment
Ideas for the Emotional Struggle
For the "Relief Buddy"
How to Help

In September 2009, when our family found out Sam had EWING SARCOMA we were all overwhelmed with questions that couldn’t be answered for months. During the past nine months, we have learned a lot and realized that all EWING patients and families can benefit from a 10-point checklist to help them get oriented and locate further information. The Sam Foundation, along with leading physicians and researchers who care for Sam, has compiled the EWING SARCOMA CHECKLIST below.

Family, Friends, and Fans along with the medical and research communities are welcome to comment further within the EWING SARCOMA topic groups at SAM'S TEAM on Facebook. A special thanks to Dr. Harvey J. Cohen, MD, PhD of Stanford University School of Medicine for editing our EWING SARCOMA CHECKLIST resource.

– Jeanne Butler
Founder, Sam Butler Callahan Foundation
aka Aunt Jeanne, and Relief Pal for Sam

#1 – WHAT’S THE CAUSE OF EWING SARCOMA? 
Answer: Full cause unknown and under research, but gene combination abnormal. It is likely people have genes that predispose them to cancer and those additional events or exposures that occur during their life interact with those genes to cause the disease. We have not identified either the predisposition genes or the specific later exposures. Most cases of EWING SARCOMA do have a specific genetic change where two chromosomes fuse to put the EWS gene next to another gene. Why this occurs, and how it causes Ewing’s Sarcoma is an area of active research.

#2 – HOW DO I FIND A DOCTOR?
Answer: Find a pediatric oncology EWING SARCOMA specialist or referral from your Doctor. It is best to establish care in a large pediatric center, as most patients with Ewing’s Sarcoma will need pediatric surgery and/or pediatric radiation. It is also best to be treated in a location where a number of children with EWING SARCOMA are treated. It is also advantageous if that institution is part of the Children’s Oncology Group (COG), as all members of COG share information across the country.

#3 – HOW IS DIAGNOSIS MADE?
Answer: Imaging studies followed by surgical biopsy EWING SARCOMA looks like other pediatric cancers under the microscope. The diagnosis is confirmed by staining for a specific marker (CD99). A special test to confirm a chromosome altercation with the EWS gene further supports the diagnosis.

#4 – WHAT ARE THE TREATMENTS FOR EWING SARCOMA?
Answer: Surgery and or radiation therapy and chemotherapy EWING SARCOMA is disease that involves the whole body. Consequently, chemotherapy is needed to treat the cells that move to other areas of the body. Radiation and/or surgery are used to treat the “primary tumor”.

#5 – WHAT DRUGS ARE USED IN TREATING EWING SARCOMA?
Answer: Combinations of drugs such as etoposide, iphosphamide, cyclophosphamide doxorubicin, vincristine, etc (perhaps others such as busulfan and melphalan) The standard treatment uses cyclophosphamide, vincristine, and doxorubicin, alternating with ifosphamide and etoposide.

#6 –WHERE SHOULD TREATMENT OCCUR?
Answer: At a Children’s Cancer Program, usually on a set protocol Treatment for EWING SARCOMA lasts a minimum of 9 months.

#7 – HOW LONG IS THE TREATMENT?
Answer: Depends on the protocol

#8 – HOW DO YOU KNOW THAT THE TREATMENT IS WORKING?
Answer: Follow with periodic CT scans and/or MRI’s as indicated

#9 – WHAT IS DONE IF THE EWING SARCOMA RECURS?
Answer: Alternate protocols are available, and experimental protocols are being investigated

#10 – WHAT ARE THE PRESENTING SYMPTOMS AND WHERE CAN IT SPREAD?
Answer: The following are the most common symptoms of EWING SARCOMA. Each child, however, may experience symptoms differently.

  • Pain around the site of a mass
  • Swelling and redness around the site of a mass
  • Fever
  • Weight loss and decreased appetite
  • Fatigue
  • Paralysis and/or incontinence (if the tumor is in the spinal region)
  • Symptoms related to nerve compression from a tumor such as numbness, tingling and pain

 

Edited by
Dr. Harvey J. Cohen, MD, PhD
Professor of Pediatrics
Pediatric Hematology/Oncology
Stanford University School of Medicine