Learn about diagnosis and treatment of this bone and soft tissue cancer, including chemotherapy, radiation, proton beam therapy and limb-saving surgery.
Ewing (YOO-ing) sarcoma is a rare type of cancer that occurs in bones or in the soft tissue around the bones.
Ewing sarcoma most often begins in the leg bones and in the pelvis, but it can occur in any bone. Less often, it starts in the soft tissues of the chest, abdomen, limbs or other locations.
Ewing sarcoma is more common in children and teenagers, but it can occur at any age.
Major advancements in the treatment of Ewing sarcoma have helped to improve the outlook for people with this cancer. After completion of treatment, lifelong monitoring is recommended to watch for potential late effects of intense chemotherapy and radiation.
Signs and symptoms of Ewing sarcoma include:
Make an appointment with your doctor if you or your child experiences any persistent signs and symptoms that worry you.
It's not clear what causes Ewing sarcoma.
Doctors know that Ewing sarcoma begins when a cell develops changes in its DNA. A cell's DNA contains the instructions that tell a cell what to do. The changes tell the cell to multiply quickly and to go on living when healthy cells would normally die. The result is a mass (tumor) of abnormal cells that can invade and destroy healthy body tissue. The abnormal cells can break away and spread (metastasize) throughout the body.
In Ewing sarcoma, the DNA changes most often affect a gene called EWSR1. If your doctor suspects that you have Ewing sarcoma, your cancer cells may be tested to look for changes in this gene.
Risk factors for Ewing sarcoma include:
Complications of Ewing sarcoma and its treatment include:
Ewing sarcoma diagnosis usually begins with a physical exam to better understand the symptoms you or your child may be experiencing. Based on those findings other tests and procedures may be recommended.
Imaging tests help your doctor investigate your bone symptoms, look for cancer and look for signs that the cancer has spread.
Imaging tests may include:
A biopsy procedure is used to collect a sample of suspicious cells for laboratory testing. Tests can show whether the cells are cancerous and what type of cancer you have.
Types of biopsy procedures used to diagnose Ewing sarcoma include:
Determining the type of biopsy needed and the specifics of how it should be performed requires careful planning by the medical team. Doctors need to perform the biopsy in a way that won't interfere with future surgery to remove the cancer. For this reason, ask your doctor for a referral to a team of experts with extensive experience in treating Ewing sarcoma before the biopsy.
A sample of your cancer cells will be tested in the lab to determine which DNA changes are present in the cells. Ewing sarcoma cells usually have changes in the EWSR1 gene. Most often the EWSR1 gene becomes fused with another gene called FLI1, creating a new gene called EWS-FLI1. Testing the cancer cells for these gene changes can help confirm your diagnosis and give your doctor clues about the aggressiveness of your disease.
Ewing sarcoma treatment usually begins with chemotherapy. Surgery to remove the cancer usually follows. Other treatments, including radiation therapy, might be used in certain situations.
Chemotherapy uses powerful drugs to kill cancer cells. Chemotherapy treatment usually combines two or more drugs that can be administered as an infusion into a vein (IV), in pill form, or through both methods.
Treatment for Ewing sarcoma usually begins with chemotherapy. The drugs may shrink the tumor and make it easier to remove the cancer with surgery or target with radiation therapy.
After surgery or radiation therapy, chemotherapy treatments might continue in order to kill any cancer cells that might remain.
For advanced cancer that spreads to other areas of the body, chemotherapy might help relieve pain and slow the growth of the cancer.
The goal of surgery is to remove all of the cancer cells. But planning the operation also takes into consideration how it will affect your ability to go about your daily life.
Surgery for Ewing sarcoma may involve removing a small portion of bone or removing an entire limb. Whether surgeons can remove all of the cancer without removing the entire limb depends on several factors, such as the size and location of the tumor and whether it shrinks after chemotherapy.
Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells.
During radiation therapy, the beams of energy are delivered from a machine that moves around you as you lie on a table. The beams are carefully directed to the area of the Ewing sarcoma in order to reduce the risk of damage to surrounding healthy cells.
Radiation therapy might be recommended after surgery to kill any cancer cells that remain. It can also be used instead of surgery if the Ewing sarcoma is located in a part of the body where surgery is not possible or would result in unacceptable functional outcomes (such as loss of bowel or bladder function).
For advanced Ewing sarcomas, radiation therapy can slow the growth of the cancer and help relieve pain.
A diagnosis of Ewing sarcoma can be frightening — especially for the family of a newly diagnosed child. With time you'll find ways to cope with the distress and uncertainty of cancer. Until then, you may find it helps to:
If there are signs and symptoms that worry you, you'll likely start by making an appointment with your primary care doctor — or the pediatrician if the concern is with your child. If your doctor suspects Ewing sarcoma, make sure you're referred to an experienced specialist.
Ewing sarcoma typically needs to be treated by a team of specialists, which may include:
Before the appointment, make a list of:
Whether you're the patient or your child is the patient, your questions might include, for example:
Your doctor will likely ask several questions. Be ready to answer them to allow more time to cover other points you want to address. Whether you're the patient or your child is the patient, the doctor may ask:
June 6th, 2020