Source: Cancer Resource Room

What is a pediatric sarcoma?
Sarcomas are cancerous tumors that grow from abnormal cells in connective tissues. Connective tissues include:

• bones
• cartilage
• muscle
• fat
• blood or lymph vessels
• nerves

Sarcomas are divided in two major groups.

Sarcomas that develop in bones and cartilage have special names, like Osteosarcoma and Chondrosarcoma. Sarcomas that develop in soft tissue (muscle, fat, blood, lymph vessels, and nerves) are called Soft Tissue Sarcomas. There are more than 30 different sarcomas. The most common types are:

• liposarcoma
• malignant fibrous histiocytoma
• leiomyosarcoma
• fibrosarcoma

Sarcomas occur in people of all ages, in both sexes, and in people who are otherwise in good health. Sarcomas usually have no known causes, except high-dose radiation or genetics. Sarcomas are rare, with only about 8,700 new cases per year in the United States. Thus, these tumors are best evaluated and treated at a cancer center by sarcoma specialists.

How are sarcomas diagnosed?
Patients may have a lump they can see or feel. It is usually a painless lump, but if it is large or pressing on nearby structures, like nerves, it can cause pain. A sarcoma can be seen or felt when it is close to the surface of the body. If it occurs deep in the body, it may grow quite large before being noticed. There are no screening tests for sarcomas.

Once a mass (or lump) is noticed, a process of evaluation begins.

Step One
The first and most important part of this process is the history of the person's illness and a thorough physical exam. X-rays are taken of the area of the mass. A CT scan or an MRI (or both) is done to help understand the size and nature of the tumor, and its relationship to the surrounding tissues. A chest X-ray or chest CT scan is done to see if disease has spread to the lungs. Blood tests help evaluate the general health of the person.

Step Two
After the preliminary tests are complete, a method of testing the tumor tissue is chosen. This is called a biopsy and can be done in one of several ways.

• Core Needle Biopsy
  If the tumor is near the surface of the skin and can be palpated (felt), this test can be performed in the office by the orthopedic or surgical oncologist. Local anesthesia will be used. If the tumor is deeper, this test will be done in the radiology suite by a radiologist. Local anesthesia and some intravenous sedative medications will be used.
• Incisional or Excisional Biopsy
  These biopsies are performed in the office or in the outpatient operating room. These types of biopsies need to be performed very carefully to avoid spreading the cancerous cells. Either local or general anesthesia will be used. In an incisional biopsy, the surgical oncologist removes a portion of the tumor. In an excisional biopsy, the surgical oncologist removes the entire tumor with little or no margin (immediate area around the tumor).

What do the results of a biopsy mean for the patient?
Two important things are learned from the biopsy.

• Type of Sarcoma
  The pathologist can decide whether or not the tumor is a sarcoma, and if so, what type it is. The particular type of sarcoma can influence decisions about the treatment.
• Grade of Tumor
  Second, something called the "grade" of the tumor is determined. Soft tissue sarcomas are graded as either low-grade or high-grade tumors. Low-grade tumors generally grow slowly and are unlikely to spread to distant sites. High-grade tumors grow more quickly and have a greater chance of spreading to distant sites. The most common site of distant spread is the lungs.

How is the treatment planned?
A number of specialists will contribute to determining an accurate diagnosis. Then a treatment plan will be made. A panel of specialists in sarcoma treatment should review the results of all the tests, radiologic studies and biopsies to create the plan. Each patient is unique so treatment should be individually designed.

• For small, low -grade tumors, treatment may involve surgery alone, or surgery and radiation.
• Large, high-grade tumors may need a combination of surgery, radiation, and chemotherapy for adequate treatment. The necessary surgery, radiation and chemotherapy differ in extent, timing, and combination for each tumor being treated.

Who will be on my treatment team?
The appropriate care of a sarcoma patient requires the expertise of people in many specialties. You may have care provided by the following specialists:

• Radiologists
• Pathologists
• Medical oncologists
• Orthopedic and general surgeons specializing in oncology
• Radiation oncologists
• Oncology nurses
• Social workers, psychiatrists or psychologists
• Physical and occupational therapists

To find out who treats sarcomas at Massachsuetts General Hospital Cancer Center >>>

Support & Education Programs

We know that being diagnosed with cancer can be stressful for you and your family. We offer a variety of cancer support services to help patients and families gain the support and information they will need to meet the challenges ahead.

To find information on the pediatric support programs, please go to Parents and Family Programs >>>

Read the most recent SUPPORT publication, a resource written by patients and families for patients and families >>>