Sarcoma: An Overview

• angiosarcoma (blood vessels
• fibrosarcoma (connective tissue)
• gastrointestinal stromal tumor (digestive system)
• Kaposi's sarcoma (skin)
• liposarcoma (fat)
• leiomyosarcoma (smooth muscle)
• malignant fibrous histiocytoma (connective tissue)
• neurofibrosarcoma (nerves)
• rhabdomyosarcoma (skeletal muscle) and
• synovial sarcoma (often near joints, but can occur anywhere).

Growth: A non-specific term that describes a collection of tissue that is growing in a way that is different from normal tissue. Growths can be benign (such as a cyst or wart) or they can be cancerous.

Tumor: A lump of tissue that is growing out of control. Tumors can be benign (not a threat to life) or they can be cancerous.

Malignant tumor: A rapidly growing tumor made up of abnormal cells.

Another term for a malignant tumor is cancer. A malignant tumor will first grow only in the area where it started. This is known as the primary tumor. The primary tumor cells may spread to other tissues or organs through the bloodstream or lymph system. Once the tumor cells have spread to other parts of the body, they will take root and start to grow other tumors. This process is called metastasizing. The new tumor name in other parts of the body is called metastatic tumor.

Radiological Imaging (X-rays and scans)

• Plain X-ray Films:
  Standard x-rays are taken to evaluate the bone. They can show tumor location. Usually more detailed imaging such as a MRI or CT scan is needed to further evaluate. Chest x-rays are also useful to evaluate for metastatic tumors in the lung.
  
• Magnetic Resonance Imaging (MRI):
  This is one of the most useful methods to image tumors in the extremities. It provides the physician with a detailed view of the tumor and its surroundings.
  
• Computed Tomography (CT scan):
  This is another way to image a tumor. Especially useful in tumors which are in the abdomen and pelvis. CT scan of the chest is also frequently recommended to assess for metastases of sarcoma, or disease which might have traveled to the lungs.
  
• Positron Emission Tomograhy (PET scan):
  This is a newer study done in Nuclear Medicine, which looks at the metabolic activity of cells in the body. Tumor cells often have very active metabolism and therefore can be seen on a PET scan. However, other conditions involving increased metabolism (including healing scars) sometimes show up on PET scans as well. PET scans are occasionally used for sarcoma patients, but usually are not as useful as CT and MRI scans.
  
• Bone Scan:
  This study is done in nuclear medicine, which uses radioactive dye to highlight the bones. This scan is sometimes used to look for cancer in other parts of the bone that has metastasized.

Biopsy
A biopsy is the only way to tell for sure if you have a malignant tumor and, if so, what kind of cancer it is. Because sarcomas are rare, a specialist in sarcoma pathology generally reviews the results of the biopsy before any treatment starts. Tumor location determines what type of biopsy will be used.

Generally two types of biopsies can be used:

• Core needle biopsy:
  This procedure is less invasive than the other method described next. It can be done in the office by your surgeon using local anesthesia. The surgeon will numb the area around the tumor and extract a small amount of tissue from the tumor with a special biopsy needle for the pathologist to examine.
• Incisional biopsy:
  A small incision (about an inch) is made, and tissue is removed for examination. This is a more invasive procedure and is most often done in the operating room under either local or general anesthesia. The advantage of this procedure is that is provides more tissue for the pathologist to review.

Surgery
Surgery is considered to be the primary treatment for bone sarcomas, and for some, is the only treatment.

The first goal of the orthopedic surgeon is to remove the tumor, along with some healthy tissue around it. This ensures that no cancer cells remain.

The second goal is to retain as much function as possible to the operative site. Sometimes manmade material is used to replace bone, artificial joints and devices are inserted and sometimes amputation is needed. The involvement of the surrounding tissue, muscle, nerves and blood vessels determine the extent to which that is possible.

Prior to your sugery, your orthopedic oncologist will discuss all your options. A thoracic surgeon performs surgery for sarcomas inside the chest or lungs (usually metastatic tumors).

RadiationTherapy
Radiation usually is administered after surgery, and is given to patients who are at risk for local tumor reoccurrence if only surgery was used for control of the tumor locally.

The goal of radiation therapy is to direct additional therapy at the tumor site to kill any remaining cancer cells that could remain after surgery.

The radiation oncologist uses your preoperative x-rays, information from your surgeon and their own treatment plan to determine the region that needs to be treated.

Radiation treatment is given daily Monday through Friday for 5-7 weeks depending on the plan discussed by your radiation oncologist.

Chemotherapy
Many factors influence the use of chemotherapy or drug treatment of sarcoma.

These factors include:

• the aggressiveness of the tumor or grade
• the size of the tumor and
• if the tumor has already spread to other areas in the body.

There are many cases in which chemotherapy is given prior to surgery to help shrink down the primary tumor so the surgeon can fully remove the tumor and maintain function of the affected area. It is also used after surgery to destroy any stray cancer cells and to prevent cancer from recurring.

Chemotherapy is often given in high doses and it can cause you to feel sick at times. The details of the chemotherapy regimen and toxicities (side effects) related to the chemotherapy will be discussed by your medical oncologist and staff. (you can also visit our web page: What to Expect: A Guide to Chemotherapy).

The chemotherapy is given to you in the outpatient clinic or in the hospital and the length of treatment is determined by which regimen your medical oncologist presents to you.