Ewing's sarcoma (also referred to as Ewing sarcoma) is an uncommon and aggressive type of cancer that originates in the bones or the surrounding soft tissues. It was first identified in 1921 by Dr. James Ewing, an American pathologist who distinguished this tumor from osteosarcoma based on how it responded to radiotherapy. Although Ewing’s sarcoma can occur at any age, it predominantly affects children and young adults, usually during puberty when rapid bone growth occurs.
What causes Ewing’s sarcoma?
Researchers have discovered that Ewing’s sarcoma forms after mutations occur in a cell’s chromosomes. In Ewing's sarcoma cells, the genetic material in chromosomes #11 and #22 is mismatched. This genetic abnormality is not inherited—instead, the chromosomal change takes place in a single cell after birth.
Researchers are still working to determine precisely what triggers the genetic abnormality that leads to Ewing’s sarcoma. Unlike other tumors that are known to arise in certain cells (e.g., breast cancer develops in breast cells), Ewing’s sarcoma is not conclusively associated with a precise cell type.
Furthermore, Ewing’s sarcoma is an uncommon disease that is not linked to behavioral or environmental risks like smoking or exposure to toxins. The only established risk factors for this cancer are being between the ages of 10 and 20 and having European ancestry, although it’s possible for anyone of any age to develop Ewing’s sarcoma.
What are the signs and symptoms of Ewing’s sarcoma?
Many children and teenagers with Ewing’s sarcoma report experiencing pain. A palpable mass may also occur, although it can take months or even years for a tumor to grow large enough to be felt or cause significant discomfort.
Ewing’s sarcoma pain may be mild and sporadic at first, then become more intense and constant over time. This cancer can develop on any bone, although around 25% of cases occur in the pelvis. Long bones such as the thighbone (femur), shinbone (tibia) and upper arm bone (humerus) are other common sites for sarcomas, followed by the chest wall and spine. Soft tissue sarcomas most often develop in the chest area and trunk.
In addition to pain and lumps just below the skin’s surface, other possible signs and symptoms of Ewing’s sarcoma include:
- Bone tenderness and swelling
- Unexplained fatigue
- A lingering fever with no known cause
- Numbness or weakness around the affected area
- Unintended weight loss
- A broken bone that occurs without an obvious cause
What is the difference between Ewing’s sarcoma and osteosarcoma?
Ewing’s sarcoma and osteosarcoma are both bone cancers that primarily occur in children and young adults. Osteosarcoma is more common and usually develops toward the ends of long bones, such as the bones that form the knee joint and shoulder joint. Ewing’s sarcoma is part of the Ewing family of tumors—which includes primitive neuroectodermal tumors (PNETs) and Askin’s tumors—and most often occurs in the pelvis, thighbone and shin bone.
Ewing’s sarcoma and osteosarcoma share many symptoms, but they derive from different cells and their treatment options vary. For example, radiation therapy is more often used to treat Ewing’s sarcoma than osteosarcoma.
How is Ewing’s sarcoma diagnosed?
Ewing’s sarcoma is sometimes detected after an injury, such as a fracture in a bone that has been weakened by a tumor. Typically, a physician will perform an exam to check for visible signs of injury or illness and ask questions about the patient’s symptoms and medical history. Based on this information, one or more of the following tests may be ordered to help rule out or diagnose Ewing’s sarcoma:
- An imaging test to view pictures of the affected bone, such as digital X-ray imaging, computerized tomography (CT), a bone scan, magnetic resonance imaging (MRI) or positron emission tomography (PET)
- A blood test to gauge kidney and liver function and check for unusual amounts of certain substances that may point to cancer
- A needle or surgical biopsy to collect a small sample of abnormal-looking cells for testing under a microscope
How is Ewing’s sarcoma treated?
The primary treatments for Ewing’s sarcoma are:
- Surgery to remove the tumor and a margin of surrounding healthy tissue
- Radiation therapy, which is often used in combination with chemotherapy to help shrink or destroy cancer cells before or after surgery
- Targeted therapy, or medications that impede the processes that fuel cancer growth
A patient’s ideal course of treatment will depend on their age, overall health and the stage and location of the tumor. Even if there is no evidence of metastasis at the time of diagnosis, an oncologist will usually plan treatment with the assumption that there is a small amount of spread. Additional treatments to help reconstruct affected bones and keep patients mobile may be necessary. Ewing’s sarcoma survival rates have significantly improved in recent years, as breakthroughs in treatment are improving prognoses and quality of life for patients.
Moffitt’s approach to Ewing’s sarcoma treatment
For the best outcomes, choose Moffitt Cancer Center first. Moffitt is ranked in the top 1% of cancer centers nationwide by experts and achieves survival rates that are up to four times higher than national averages. The multispecialty team in Moffitt’s Sarcoma Program focuses exclusively on sarcomas and spearheads ambitious clinical trials that are steadily improving outcomes. Thanks in part to our robust research efforts, Moffitt has been designated as a Comprehensive Cancer Center by the National Cancer Institute. We are the only such center based in Florida.
If you would like to consult with a Moffitt specialist about potential Ewing’s sarcoma symptoms or receive a second or third opinion, call our team today at 1-888-663-3488 or complete a new patient registration form online. A referral is not necessary to visit Moffitt. Your well-being and peace of mind are our top priorities, which is why you’ll be connected to an expert as soon as possible.