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Sarcoma

The term "sarcoma" applies to a group of rare cancers that arise from the bone and the soft tissues that are referred to collectively as "connective tissues."

Soft tissue sarcomas can arise in fat, skeletal and smooth muscle, blood vessels, deep skin tissues, and nerves.

The American Cancer Society estimates that approximately 8,700 new soft tissue sarcomas were diagnosed in the United States during 2001. Of these, slightly over half occur in males and less than half in females. Sarcomas of the soft tissues occur more commonly in adults tan in children, although exceptions do occur.

Sarcomas of the bone arise from the tissues that comprise the bones, including bone itself, cartilage, and some supporting cells that are found within the bones. Most of the sarcomas that arise in the soft tissues have a counterpart that arises as a primary tumor in the bones as well.

Most of the cancers that are present in bones are not sarcomas or primary tumors. Instead, most of the cancers that are discovered in the bones have spread to the bones from another site, such as the breast, prostate, colon, lung and kidney. The process of spreading from the site of origin to another distant site is known as metastasis. When cancer spreads to the bones, it is not referred to as bone cancer, but is referred to as metastatic cancer in bone.

Each year, approximately 2,000 bone sarcomas are diagnosed in the United States. Of these, the most common tumor (approximately 900 cases) is capable of producing tissue that is called osteosarcoma, which develops most commonly in teenagers and young adults. The second most common bone sarcoma is chondrosarcoma, which makes a form of cartilage and occurs mot frequently in adults.

Types of sarcoma:

The following are some of the more common forms of sarcoma and the tissue from which they are thought to develop:

  • Angiosarcoma - blood or lymphatic vessels
  • Chrondrosarcoma - cartilage tissue
  • Ewing's sarcoma - cells of primitive nerve epithelial tissues
  • Fibrosarcoma- fibrous tissue that forms tendons and ligaments
  • Kaposi's sarcoma - blood vessels and supporting cells
  • Leiomyosarcoma - smooth muscle (involuntary) cells
  • Liposarcoma - fat cells and tissue
  • Malignant fibrous histocytoma - most common soft tissue tumor found in arms or legs of adults
  • Malignant peripheral nerve sheath tumor, sometimes called neurofibrosarcoma, malignant Schwannoma or neurogentic sarcoma - cells that support and protect nerve cells
  • Multiple myeloma - plasma cells, cells that make antibodies
  • Osteosarcoma - bone-forming cells
  • Rhabdomyosarcoma - skeletal muscle
  • Synovial sarcoma - connective tissue cells near a joint

Who is at risk for sarcoma?

Certain factors have been associated with an increased risk for developing sarcoma. These include:

Family History
Certain inherited conditions increase the risk of developing sarcoma, such as:

  • Neurofibromatosis: a condition characterized by benign tumors, which develop under the skin nerves and in other parts of the body. About 5% of people with one or more of these tumors will develop malignant peripheral nerve sheath tumors.
  • Gardner's syndrome: a syndrome that can cause benign polyps and cancers in the intestine and may cause a tumor in the fibrous tissues of the abdomen.
  • Li-Fraumeni syndrome-a condition that can increase the risk of developing breast cancer, brain tumors, leukemias, as well as soft tissue and bone sarcomas.
  • Retinoblastoma-this inherited form of eye cancer in children may increase the risk of developing soft tissue sarcoma or bone sarcoma
  • Multiple hereditary osteochondromas-having these benign bone tumors formed by bone and cartilage may increase the risk of developing osteosarcoma

Damaged Lymph Nodes
When lymph nodes have been damaged by radiation therapy or have been removed, angiosarcoma – a cancer of lymph vessels – can develop. Chronic lymphedema, a condition in which excess fluid called lymph collects in tissues and causes swelling in an extremity, can also cause angiosarcoma.

Noncancerous Bone Diseases
Individuals with certain non-cancerous diseases such as Paget's disease – a benign condition affecting one or more bones – and multiple hereditary ostestechondromas (see above) are at an increased risk for developing osteosarcoma as adults.

Radiation
Individuals exposed to high doses of radiation for treatment of other cancers are at a slightly higher risk for soft tissue sarcoma (less than 5%, according to the American Cancer Society) and osteosarcoma. Exposure to high doses of radium can be a risk factor for bone sarcoma.

Chemicals
Exposure to certain chemicals, such as dioxin and herbicides containing high doses of phenoxyacetic acid, may put people at risk for soft tissue sarcomas.

Warning Signs

Soft tissue sarcomas
Only about 50% of soft tissue sarcomas are noticed at an early stage. The appearance of a new lump or one that is growing, which may or may not be painful warrants immediate medical attention.

Sarcoma of the bone
Warning signs for sarcoma of the bone may include:

  • Persistent pain in the back, arm or leg that is present when at rest rather than during activity
  • A painless or painful mass in the arm, leg, hip or spine area

What causes sarcoma?

The precise cause of most sarcomas is not known. However, there are certain risk factors that make sarcomas more likely to develop. Inherited conditions mentioned above, such as neurofibromatosis, Gardner's syndrome, and Li-Fraumeni syndrome, may increase the risk of developing certain sarcomas because they involve mutations in DNA, causing normal cells in tissues to become cancerous. Exposure to high levels of radiation or cancer-causing chemicals may also cause DNA mutations and, as a result, sarcomas.

Early Detection and Screening

Routine screenings tests for sarcoma are not currently available and symptoms of soft tissue sarcomas may not appear until the disease is in an advanced stage. Those adults who are at a greater risk because of a family history of sarcoma should consider gene testing. Anyone who detects unexplained lumps - especially accompanied by pain that occurs while resting - or other warning signs, should immediately contact their physician.

Diagnosis

The diagnostic process for determining sarcoma begins with the patient's family medical history for risk factors and a close examination of the lump, if one exists, to determine its size, shape and impact on surrounding areas. Further testing, as described below, will be recommended to confirm the presence of a cancerous tumor and gather important information for staging, which categorizes the cancer's spread.

There are four stages of sarcoma, determined by how aggressive (the "grade") the cancer cells appear, the tumor's size, and whether the cancer cells have spread (metastasized) to lymph nodes or other parts of the body. This information is critical for determining the appropriate treatment. The higher the stage, the more advanced the cancer.

A stage 1A or 1B tumor closely resembles the normal cells in which the cancer has started and has not spread.

Stage 4A or 4B tumors are relatively large, contain aggressive cancer cells, and have metastasized to lymph nodes in the immediate area or to other parts of the body.

The Ewing's family of tumors is only staged as either a localized disease (found in only one place) or as a metastasized disease.

Physicians at NewYork-Presbyterian's Cancer Centers use the latest technologies to diagnose sarcomas and guide treatment recommendations. These include:

  • Ultrasound. This painless procedure uses sound waves to determine whether the tissue is normal or whether a tumor is present.
  • Computed Tomography (CT Scan). This procedure takes pictures of the body from different angles, which are then combined in a computer to produce a detailed, cross-sectional picture of the inside of the body. It will help detect the presence of a mass or enlarged lymph nodes, which could denote more advanced disease.
  • Magnetic Resonance Imaging (MRI). Using large magnets and radio waves, this procedure also produces computer-generated cross-sectional pictures of the inside of the body. The diagnostic images are similar to a CT scan, but provide greater detail.
  • Bone Scan. Using a small amount of radioactive material that, when injected into the bloodstream, collects in the bones. Abnormal deposits are detected by special instrument called a scanner, which records images of the bone on a special film for viewing.
  • Fine Needle Aspiration. Using a very thin needle, tiny fragments of tissue are removed from the tumor mass. A CT scan may be used to guide the needle during this non-surgical procedure. This technique is helpful in indicating if the mass is something other than a sarcoma, such as an infection or a benign tumor.
  • Core Needle Biopsy. This technique is used to remove more tissue than in a fine needle aspiration and is used to tell whether a sarcoma is present or not.
  • Excisional or Incisional Biopsy. With these types of biopsies, the surgeon removes the entire tumor (excisional) or part of the tumor (incisional) to diagnose the exact type and grade of sarcoma. These procedures are performed in the hospital using local anesthesia if the tumor is close to the skin surface or general anesthesia if the tumor is deep inside the body.
  • Infrared Analysis. Pathologists and orthopedic oncologists in the Columbia Weill Cornell Cancer Centers are using this type of analysis to identify specific markers to predict cancerous bone tumor activity and spread.

Treatment of sarcomas:

NewYork-Presbyterian's Cancer Centers are at the forefront of treatment for soft-tissue sarcoma and sarcoma of the bone, including limb salvage surgery, prosthetic reconstruction and individualized rehabilitation programs following removal of tumors of the limbs, as well as a new percutaneous technique for treating pathological crush fractures of the vertebral body in patients with round cell malignancies, most notably lymphomas and myelomas. The results have been outstanding in correcting the fracture and angle of the spine while providing dramatic pain relief. This technique is currently used to treat patients suffering from fractures secondary to round cell malignancies, as well as those who have secondary osteoporosis due to steroids or chemotherapy. In addition, the Cancer Centers radiation oncologists are among the leaders in developing limb-preserving techniques using brachytherapy (see below) in the treatment of extremity soft tissue sarcomas with outstanding results. Our physicians also have extensive experience with cryosurgery to extend margins without sacrificing tissue, such as bone, adjacent to soft tissue sarcoma.

Following is information about the treatment of sarcomas with surgery, radiation therapy and chemotherapy, often used in combination and based on the type, location and stage of the tumor.

Surgery
is the most common treatment of sarcoma in which the tumor is removed along with a border of healthy tissue. Surgery is more difficult if the tumor is in the abdomen and next to vital organs. Most sarcomas of the extremity (arm or leg) can be removed with limb-preserving surgery. Amputation may be required rarely on patients when essential nerves, muscles or arteries are vulnerable to sarcoma cells. Lymph nodes are removed if the cancer has spread. If a sarcoma has metastasized, addition treatment is indicated. If the metastatic sarcoma appears only in the lung, the tumor may be able to be surgically removed.

Radiation
This therapy uses high-energy radiation to kill cancer cells. Radiation therapy may be used as the primary treatment in patients who are not healthy enough to undergo surgery, or as an adjuvant (additional) therapy to shrink a tumor before surgery or to destroy the remaining cancer cells following surgery. External beam radiation therapy delivers radiation to the cancerous site from a source outside the body, while brachytherapy involves surgically placing a small pellet of radioactive material directly into the cancerous cells.

Chemotherapy
Chemotherapy for treating sarcomas generally involves a combination of several anticancer drugs. Chemotherapy can be used to shrink the tumor before surgery or given in addition to surgery to eliminate remaining cancer cells. In some cases, it is the primary treatment - especially when the tumor cannot be surgically removed. The drugs are delivered intravenously or may be taken orally. Occasionally, the drugs are injected into the blood vessels near the tumor site, which is called regional chemotherapy.

Biological Therapy
Used in the treatment of Kaposi's sarcoma, biological therapy involves chemicals produced by the body or in a laboratory to boost the immune system's ability to destroy cancer cells.

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