Sarcoma

What it is

Sarcomas are a diverse group of malignant tumors that develop from fat, muscles, nerves, joints, blood vessels, bones, and deep skin tissues. Sarcomas are difficult to differentiate from other malignancies when they are found within organs; thus, they are frequently misdiagnosed and highly underreported.

Soft tissue sarcoma and osteosarcoma (bone sarcoma) incidence rates have remained relatively constant over the past 30 years; however, soft tissue sarcoma is more deadly due to the lack of detectable symptoms at early disease stages. Several subtypes of osteosarcoma and soft tissue sarcoma exist.

  • Ewing sarcoma family of tumors is a group of tumors that form from a certain kind of cell in bone or soft tissue. In some patients, the tumor may have spread by the time it is diagnosed. Ewing tumors usually occur in teenagers and are more common in boys and Caucasians.
     
  • Kaposi sarcoma is a disease in which malignant (cancer) cells form in the tissue lining the lymph vessels under the skin or in mucous membranes. Kaposi sarcoma is different from other cancers in that lesions may begin in more than one place in the body at the same time. Classic Kaposi sarcoma is found most often in older men of Italian or Eastern European Jewish origin.
     
  • Adult soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body. There are many types of soft tissue sarcoma. The cells of each type of sarcoma look different under a microscope, based on the type of soft tissue in which the cancer began.
     
  • Uterine sarcoma is a very rare kind of cancer that forms in the uterine muscles or in tissues that support the uterus. Uterine sarcoma is different from cancer of the endometrium, a disease in which cancer cells start growing inside the lining of the uterus.
     
  • Osteosarcoma and malignant fibrous histiocytoma (MFH) of the bone are diseases in which malignant (cancer) cells form in bone. Osteosarcoma is the most common type of bone cancer. Malignant fibrous histiocytoma (MFH) of bone is a rare tumor of the bone. It is treated like osteosarcoma. Osteosarcoma is most common in teenagers and young adults. In children and teenagers, it often develops around the knee.

 

What to look for

Ewing sarcoma
Possible signs of Ewing sarcoma family of tumors include swelling and pain near the tumor. These and other symptoms may be caused by Ewing sarcoma family of tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Pain and/or swelling, most commonly in the arms, legs, chest, back, or pelvis (area between the hips)
  • A lump (which may feel warm) in the arms, legs, chest, or pelvis
  • Fever for no known reason
  • A bone that breaks for no known reason

Classic Kaposi sarcoma
Symptoms of classic Kaposi sarcoma may include slow-growing lesions on the legs and feet. Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions may form in other parts of the body, such as the stomach, intestines, or lymph nodes. The lesions usually don't cause any symptoms, but may grow in size and number over a period of 10 years or more. Pressure from the lesions may block the flow of lymph and blood in the legs and cause painful swelling. Lesions in the digestive tract may cause gastrointestinal bleeding.

Adult soft tissue sarcoma
Possible signs of adult soft tissue sarcoma include a lump or swelling in soft tissue of the body. A sarcoma may appear as a painless lump under the skin, often on an arm or a leg. Sarcomas that begin in the abdomen may not cause symptoms until they become very large. As the sarcoma grows larger and presses on nearby organs, nerves, muscles, or blood vessels, symptoms may include:

  • Pain
  • Trouble breathing

Other conditions may cause the same symptoms that soft tissue sarcomas do. A doctor should be consulted if any of these problems occur.

Uterine sarcoma
Possible signs of uterine sarcoma include abnormal bleeding. Abnormal bleeding from the vagina and other symptoms may be caused by uterine sarcoma. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Bleeding that is not part of menstrual periods
  • Bleeding after menopause
  • A mass in the vagina
  • Pain or a feeling of fullness in the abdomen
  • Frequent urination

Osteosarcoma and MFH
Possible signs of osteosarcoma and MFH include pain and swelling over a bone or a bony part of the body. These and other symptoms may be caused by osteosarcoma or MFH. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Swelling over a bone or bony part of the body
  • Pain in a bone or joint
  • A bone that breaks for no known reason


How we find it

The following tests and procedures may be used to diagnose the various types of sarcomas:

  • Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
     
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:

    • The number of red blood cells, white blood cells, and platelets.
       
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
       
    • The portion of the blood sample made up of red blood cells. 
       
  • Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances, such as lactate dehydrogenase (LDH), released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
     
  • Sedimentation rate: A procedure in which a sample of blood is drawn and checked for the rate at which the red blood cells settle to the bottom of the test tube.
     
  • X-ray: An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
     
  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
     
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
     
  • Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
     
  • Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
     
  • PET scan (Positron Emission Tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
     
  • Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
     
  • Endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease. This is used to find Kaposi sarcoma lesions in the gastrointestinal tract.
     
  • Bronchoscopy: A procedure to look inside the trachea and large airways in the lung for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
     
  • Incisional biopsy: The removal of part of a lump or a sample of tissue.
     
  • Core biopsy: The removal of tissue using a wide needle.
     
  • Pelvic exam: An exam of the vagina, cervix, uterus, fallopian tubes, ovaries, and rectum. The doctor or nurse inserts one or two lubricated, gloved fingers of one hand into the vagina and the other hand is placed over the lower abdomen to feel the size, shape, and position of the uterus and ovaries. A speculum is also inserted into the vagina and the doctor or nurse looks at the vagina and cervix for signs of disease. A Pap test or Pap smear of the cervix is usually done. The doctor or nurse also inserts a lubricated, gloved finger into the rectum to feel for lumps or abnormal areas.
     
  • Pap test: A procedure to collect cells from the surface of the cervix and vagina. A piece of cotton, a brush, or a small wooden stick is used to gently scrape cells from the cervix and vagina. The cells are viewed under a microscope to find out if they are abnormal. This procedure is also called a Pap smear. Because uterine sarcoma begins inside the uterus, this cancer may not show up on the Pap test.
     
  • Dilatation and curettage: Surgery to remove samples of tissue or the inner lining of the uterus. The cervix is dilated and a curette (spoon-shaped instrument) is inserted into the uterus to remove tissue. Tissue samples may be taken and checked under a microscope for signs of disease. This procedure is also called a D&C.
     
  • Endometrial biopsy: The removal of tissue from the endometrium (inner lining of the uterus) by inserting a thin, flexible tube through the cervix and into the uterus. The tube is used to gently scrape a small amount of tissue from the endometrium and then remove the tissue samples. A pathologist views the tissue under a microscope to look for cancer cells.

 

How we treat it

 Five types of standard treatment are used to treat sarcomas:

  1. Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type of the cancer being treated and whether it is found at the place it first formed only or whether it has spread to other parts of the body.
     
  2. Surgery is usually done to remove cancer that is left after chemotherapy or radiation therapy. When possible, the entire tumor is removed by surgery. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body or a donor, or with an implant such as artificial bone.

    Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy.
     
  3. Radiation therapy may be used to shrink the tumor before surgery so less tissue needs to be removed. It may also be used to kill tumor cells that are left after surgery or chemotherapy. Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type of the cancer being treated and whether it is found at the place it first formed only or whether it has spread to other parts of the body.
     
  4. Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent used to treat Kaposi sarcoma.
     
  5. Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy is used to reduce the production of hormones or block them from working.

 

Source: National Cancer Institute (http://www.cancer.gov/)