Ewing’s Sarcoma

What Is Ewing’s Sarcoma?

Ewing’s sarcoma is a cancer that occurs primarily in the bone or soft tissue. Ewing’s sarcoma can occur in any bone, but is most often found in the long bones such as the femur (thigh), tibia (shin), or humerus (upper arm). It can involve the muscle and the soft tissues around the tumor as well.

Ewing’s sarcoma cells can also spread (metastasize) to other areas of the body including the bone marrow, lungs, kidneys, heart, adrenal gland, and other soft tissues. This type of bone tumor accounts for 16% of bone cancers, reports the American Cancer Society.

Who develops Ewing’s sarcoma?

Ewing’s sarcoma is a very rare cancer in adults. However, Ewing’s sarcoma most often occurs in children between the ages of ten and 20, accounting for about 1–2% of childhood cancers. Prior to adolescence, the number of males and females affected are equal. After adolescence, however, the number of males affected is slightly higher than the number of females. It has been suggested that the increased rate of growth among males during adolescence may account for this increased incidence.

Ewing's Sarcoma

Long-term outlook for Ewing’s sarcoma

Prognosis for Ewing’s sarcoma greatly depends on:

  • The extent of the disease
  • The size and location of the tumor
  • Presence or absence of metastasis
  • The tumor’s response to therapy
  • Your age and overall health
  • Your tolerance of specific medications, procedures, or therapies

As with any cancer, prognosis and long-term survival can vary greatly from person to person. Every individual is unique and treatment and prognosis is structured around your needs. Prompt medical attention and aggressive therapy are important for the best prognosis.

Continuous follow-up care is essential for a person diagnosed with Ewing’s sarcoma. Late effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of Ewing’s sarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.

Ewing’s Sarcoma Symptoms

The following are the most common symptoms of Ewing’s sarcoma. However, each individual may experience symptoms differently. Symptoms may include:

  • Pain around the site of the tumor
  • Swelling and/or redness around the site of the tumor
  • Fever
  • Weight loss, decreased appetite
  • Fatigue
  • Paralysis and/or incontinence (if the tumor is in the spinal region)
  • Symptoms related to nerve compression from tumor (e.g., numbness, tingling, paralysis, etc.)

The symptoms of Ewing’s sarcoma may resemble other medical conditions or problems. Always consult your doctor for a diagnosis.

Diagnosis of Ewing’s Sarcoma

Examination by your doctor

You should report any symptoms of Ewing’s sarcoma to your doctor. Symptoms may be caused by cancer or by other, less serious problems. To find out what is causing your symptoms, your doctor will ask you about these things.

  • Your health history
  • Your family’s history of cancer
  • Your lump, if you have one
  • Your pain, if you have any

Your doctor will also perform a careful physical exam. If you have a lump, the doctor will look at its size and shape of the lump and the effect it has had on surrounding areas.

Initial tests for Ewing’s sarcoma

The doctor may also schedule one or more tests.

X-ray

X-rays

Doctors can identify most Ewing’s tumors with an X-ray. An X-ray uses very low doses of radioactive rays to see through your skin. It imprints an image of the inside of your body onto a piece of film. Sometimes doctors skip the X-rays and go right to other tests such as a computed tomography scan (CT scan) or magnetic resonance imaging (MRI) scan.

CT scan

Computed tomography scan (CT scan)

A CT scan can help your doctor identify more clearly a Ewing’s tumor. A CT scanner takes X-rays of your body from different angles as you slide through it on a table. A computer combines these pictures to produce a more complete picture of the inside of your body.

MRI

Magnetic resonance imaging (MRI)

An MRI can more clearly define abnormalities to see if they are really due to a Ewing’s tumor. During an MRI, large magnets and radio waves produce detailed pictures of the inside of your body. The energy from the radio waves creates patterns formed by different types of tissue and diseases. This produces cross-sectional pictures that look like slices of your body. Your doctor may order an MRI of a certain area of bone if you are having a lot of pain at a certain spot. These pictures will also help him or her see what part of a lump to take out in a biopsy.

For this test, you lie still on a table as it passes through a tube-like scanner. The scanner directs a continuous beam of radio waves at the area being examined. A computer uses data from the radio waves to create pictures of the inside of your body. You may need more than one set of images.

Bone scan

Bone Scan

A bone scan is a specialized radiology procedure used to examine the various bones of the skeleton to identify areas of physical and chemical changes in bone. Bone scans are used primarily to detect the spread of metastatic cancer.

For this test, a nurse or technician injects you with radioactive dye and then takes scans of your bones. The picture shows areas of cells where the radiation is concentrated, which can mean that there is an abnormality. It does not necessarily mean there is a tumor. Other things, such as arthritis or bone fractures, can also make a bone scan abnormal. Your doctor may also use this test to find out if a bone tumor has spread to other bones or if a soft tissue tumor has spread to bone.

PET scan

Positron emission tomography (PET) scan

This test is similar to a bone scan, but a slightly radioactive sugar is injected into the blood. Tumors use more of this sugar than normal cells, so the radiation collects in tumors, where it can be seen with a special camera. PET scans can help determine the spread of Ewing’s tumors, find out if abnormal areas seen on a bone scan or CT scan are tumors, and help follow the response of the tumor to treatment.

These tests can help show where the tumor is and whether it has spread. They may give enough information for a doctor to tell the cause of the problem, or you may need further testing.

Blood test

What Is a Blood Test?

Your evaluation may include the performance of specific blood tests, including (but not limited to) cell counts, measurement of various blood chemistries and markers of inflammation. We can measure many things in your blood such as salts, blood cell counts and protein markers specific to the heart (one is called BNP). Additional tests may include blood chemistries, evaluation of liver and kidney functions, and genetic studies). In some cases, genetic testing may be recommended. In addition, we may ask you to join our ongoing studies by allowing us to collect and store some of your blood for future analysis.

Biopsy

Biopsy

In some cases, the doctor will remove a sample of tissue from your tumor. This is called a biopsy.  A doctor who specializes in identifying disease, called a pathologist, will look at the sample under a microscope to check for cancer.

Your doctor may send you to a surgeon or another doctor for a biopsy. Although a physical exam and the pictures of the growth may suggest that a tumor is cancerous, a biopsy is the only way to be certain. The biopsy tells the doctor what, if any, type of tumor you have. Your doctor might perform one of these three different types of biopsy. If there is a chance that a tumor is a Ewing’s tumor, it is very important that the biopsy be done by a doctor with experience in treating this disease.

Types of biopsies:

  • Excisional and incisional biopsy
  • Needle biopsy
  • Bone marrow aspiration biopsy

Excisional and incisional biopsy

Excisional Biopsy and Incisional Biopsy

When the entire tumor is removed, the procedure is called an excisional biopsy. If only a portion of the tumor is removed, the procedure is referred to as an incisional biopsy. When possible, excisional biopsy is the preferred method when melanoma is suspected.

An excisional biopsy, also called a wide local incision, involves surgical removal of a tumor and some normal tissue around it. The amount of normal tissue taken (also called the clinical margin) depends on the thickness of the tumor. In the case of possible melanoma, skin grafting (taking skin from another part of the body to replace the skin that is removed) or rotation flaps of skin from other sites may be used to cover the wound resulting from the wide local excision, but most cutaneous melanoma excisions can be closed without placement of a skin graft.

Needle biopsy

Needle Biopsy

Needle biopsy, also called image-guided biopsy, is usually performed using computed tomography (CT), real-time X-ray (fluoroscopy), ultrasound, or magnetic resonance (MR) to guide the procedure. In the most difficult cases, needle biopsies are performed with the aid of equipment that creates a computer-generated image and allows radiologists to see an area inside the body from various angles. This “stereotactic” equipment helps them pinpoint the exact location of the abnormal tissue and to avoid injuring normal tissue.

Biopsies may involve techniques called “fine needle biopsy” or “core biopsy,” depending on the amount of material needed, the consistency of the tissue, and whether infection or other diagnoses are being considered. Both require use of long skinny needles, skinnier than an IV. Most are performed with local anesthetic and sedation only without the need for general anesthesia. Patients go home with nothing more than a band-aid.

Needle biopsy is typically an outpatient procedure with very infrequent complications; less than 1 percent of patients develop bleeding or infection. In over 90 percent of patients, needle biopsy provides enough tissue for the pathologist to arrive at a diagnosis.

The doctor inserts a thin needle through your skin to remove tiny bits of tissue from the tumor. This type of biopsy does not require surgery, and you are partially awake during this test. The doctor may use a CT scan to guide the needle. Sometimes, this test does not remove enough tissue to know whether the lump is cancer, but it can tell whether it is something else, such as an infection.

  • MRI-directed core needle biopsy
  • Needle wire localization

Needle biopsy facts:

  • The abnormality can be biopsied while important nearby structures such as blood vessels and vital organs can be seen and avoided.
  • The patient is spared the pain, scarring and complications associated with open surgery.
  • Recovery times are usually short and patients can quickly resume normal activities within a day.

Bone marrow aspiration

Bone Marrow Aspiration and Biopsy

Bone marrow aspiration and biopsy involves the removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. This type of biopsy is performed either from the sternum (breastbone) or the iliac crest hipbone (the bone area on either side of the pelvis on the lower back area). The skin is cleansed and a local anesthetic is given to numb the area. A long, rigid needle is inserted into the marrow, and cells are aspirated for study; this step is occasionally uncomfortable. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. A core biopsy (removing a small bone ‘chip’ from the marrow) may follow the aspiration.

Bone marrow aspiration biopsy

Bone Marrow Aspiration and Biopsy

Bone marrow aspiration and biopsy involves the removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. This type of biopsy is performed either from the sternum (breastbone) or the iliac crest hipbone (the bone area on either side of the pelvis on the lower back area). The skin is cleansed and a local anesthetic is given to numb the area. A long, rigid needle is inserted into the marrow, and cells are aspirated for study; this step is occasionally uncomfortable. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer. A core biopsy (removing a small bone ‘chip’ from the marrow) may follow the aspiration.

Ewing’s sarcoma is difficult to distinguish from other similar tumors. Diagnosis is often made by excluding all other common solid tumors, and by the use of genetic studies.

Staging of Ewing’s Sarcoma

What is staging?

Doctors use staging to find out if cancer has spread. It is important to know the stage of your cancer so that your doctor can decide what kind of treatment to recommend.

Even if the cancer has spread to another part of your body, it’s not called a new cancer. If Ewing’s sarcoma spreads to your lungs, it’s not considered lung cancer. It’s called metastatic Ewing’s sarcoma.

Staging system for Ewing’s sarcoma

Ewing’s sarcomas of the bone can be staged with the same detailed staging system that is used for other types of bone cancers, which is known as the American Joint Commission on Cancer TNM staging system. However, for practical purposes (including deciding on treatment), doctors use a simpler system, dividing Ewing’s tumors into the following two groups:

  • Localized cancer: This is cancer that has not spread. The tumor remains in the tissue where it developed or in nearby tissues, such as muscle or tendon.
  • Metastatic cancer: This cancer has spread from where it started to other parts of the body, such as the lungs, other bones, or bone marrow. Less often it spreads to the lymph nodes or the liver.
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