Question

An 11-year-old boy presents to the emergency department with low back pain and a palpable mass on his right side near his hip. His parent also reports intermittent, low grade fever and weight loss over the past 3 months. After a careful physical examination, laboratory studies, and imaging studies, a diagnosis of Ewing sarcoma is determined.

1. Outline the process that has most likely occurred in his body.

2. What would you expect for clinical manifestations?

3. What diagnostic tests were used and what do these tell you?

4. What treatment measures would you anticipate?

Answer 1

1) 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's sarcoma that has spread from the initially affected bone to one or more sites in the body, distant from the site of origin, is called metastatic. The most common site to which Ewing's sarcoma spreads, or metastasizes, is the lungs.

2)

Symptoms

When you have Ewing’s, you may feel pain, swelling, or stiffness in the area of the tumor (arms, legs, chest, back, or pelvis) for weeks or months. This could get mistaken for bumps and bruises. In children, you might mistake it for sports injuries.

Other symptoms include:

● A lump near skin that feels warm and soft to the touch

● Constant low fever

● Limping because your legs hurt

● Bone pain that gets worse when you exercise or during the night

Broken bones without an obvious cause

● Weight loss

● Always being tired

● Paralysis or loss of bladder control if the tumor is near your spine

3)

X-rays: These take an image of a certain area of your body where a tumor is suspected. If the X-rays show any problems, your doctor may order other imaging tests.

Bone scan: This is used to find out whether cancer cells are in your bones. A small amount of radioactive dye is injected into one of your veins. The dye will collect in the bones that have tumors. When you lie beneath a bone scanner, the doctor will be able to detect where the radioactive dye has gathered.

Magnetic resonance imaging (MRI): For this test, you lie flat and still on a surface that slides you inside the tube-shaped MRI machine. It uses magnetic field and radio waves to create a detailed, 3-D image inside your body.

Computed tomography scan (CAT/CT scan):Like you would do for an MRI, you lie flat inside a CT scanner, which takes X-ray pictures while linked to a computer. The MRI is generally preferred over the CT scan.

Positron emission tomography scan ( PET scan): You lie inside the PET scanner while a technician injects a small amount of radioactive sugar into a vein. The cancer cells show up brighter in the scan because they use more sugar than healthy cells.

Blood test: This cannot confirm Ewing’s sarcoma. But a complete blood count, or CBC, checks how many red blood cells, white blood cells, and how much hemoglobin (a protein that carries oxygen) you have.

Biopsy: Your doctor removes a piece of your tissue with either a needle or during surgery. That sample is viewed under a microscope to see if it’s cancerous. This can confirm whether you have Ewing’s sarcoma.

Bone marrow aspiration and biopsy: Your doctor numbs an area of your body, usually in the hips. She inserts a needle and takes a small piece of bone marrow .

4)Treatments

Treatment options include:

Chemotherapy: This is usually the first step. With this option, medicines are used to kill cancer cells and stop them from growing. They can be injected into your bloodstream. Your doctor may use more than one type of chemotherapy at a time or combine this with surgery and radiation.

Surgery: Your doctor will try to remove the tumor to stop its spread. In some cases, she may have to amputate an arm or leg if the tumor has spread a lot.

Radiation: In this therapy, a technician will use X-rays and other types of radiation to kill the cancer cells. This can be done using machines outside the body to deliver the dose, or through needles and tubes sent right to the tumor.