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Soft Tissue Sarcoma
  Cancer, Sarcoma, Soft Tissue
 Contributing risk factors
 Conditions suggested by it
 Prevention
 


Soft tissue sarcoma is a disease in which cancer cells are found in soft tissue in the body. Soft tissue includes muscles, tendons, connective tissues, fat, blood vessels, nerves, and synovial (joint) tissues. There are many types of soft tissue sarcoma. The term soft tissue is used to distinguish these sarcomas from bone sarcomas. The types of tumor that can develop from different soft tissues include:

Incidence; Causes & Development; Risk Factors
Soft tissue sarcomas are relatively uncommon. Although they may be found in children, soft tissue sarcomas are more common in adults.

They can develop in any part of the body. Half of them develop in the arms or legs. The rest arise in the trunk, head and neck area, internal organs, or the retroperitoneum (the back of the abdominal cavity).

Risk factors include the following:
  • Exposure to certain types of chemicals, such as herbicides, wood preservatives, polycyclic hydrocarbons, dioxin
     
  • Exposure to ionizong radiation, including therapeutic, diagnostic, and accidental. This risk factor accounts for under 5% of sarcomas. The most common cause of radiation exposure in patients who develop sarcomas is from radiation given to treat other tumors, such as breast cancer or lymphoma. The average time between radiation exposure and diagnosis of a sarcoma is about 10 years.
     
  • A history of angiosarcoma of the liver
     
  • A weak or poorly-functioning immune system
     
  • Inherited diseases such as:
    • Neurofibromatosis - a disease that usually runs in families and is characterized by many neurofibromas (benign tumors that form in nerves under the skin and in other parts of the body). One or more neurofibromas will develop into a malignant peripheral nerve sheath tumor in about 5% of people with neurofibromatosis.
    • Gardner's syndrome - a disease that runs in families and leads to benign polyps (precancerous growths) and cancers in the intestines. It also causes desmoid tumors (a type of low-grade fibrosarcoma) in the abdomen and benign bone tumors.
    • Li-Fraumeni syndrome, which runs in families and increases the risk of developing breast cancer, brain tumors, leukemias, and cancer of the adrenal glands. People with Li-Fraumeni syndrome also have an increased risk of developing soft tissue sarcomas and bone sarcomas. If their cancer is treated with radiation, they have a very high chance of developing a new cancer in the part of the body that received the radiation.
    • Retinoblastoma - an eye cancer of children that can be hereditary. Children with the inherited form of retinoblastoma also have an increased risk of developing bone or soft tissue sarcomas.

     
  • Damaged lymph nodes: Lymph (a clear fluid containing immune system cells) is transported throughout the body by lymph vessels and filtered by lymph nodes. Lymphangiosarcomas, a cancer of lymph vessels, can develop in parts of the body where lymph nodes have been removed by surgery or damaged by radiation therapy. Although this is a rare complication, it affects some women whose axillary lymph nodes were removed and who received radiation to treat their breast cancer.
Signs & Symptoms
In the early stages, a sarcoma is small and does not produce symptoms. As the tumor grows, it may push aside normal body structures, causing symptoms.

The most common symptom of a sarcoma is a lump or swelling that may or may not be painful. Symptoms vary, depending on the part of the body that is affected. For example, tumors found in the following areas of the body may develop these symptoms:Diagnosis & Tests
The doctor may decide to order an X-ray or other tests if a tumor is suspected. However, the only way to confirm the diagnosis is with a biopsy. If a sarcoma is found, staging tests are performed to find out if the cancer has spread and, if so, to what extent.

Treatment & Prevention
Treatment depends on the stage of the cancer. Conventional treatments may include:
  • Surgery - surgical removal of the cancerous tumor and nearby tissues, and possibly nearby lymph nodes.
  • Radiation Therapy (Radiotherapy) - the use of radiation to kill cancer cells and shrink tumors. When a sarcoma is aggressive looking, and the surgeon has completed his or her task of removing as much of it as possible, adding radiation will significantly reduce the chances of the cancer coming back. Radiation may be:
    • External Radiation Therapy - radiation directed at the tumor from a source outside the body
    • Internal Radiation Therapy - radioactive materials placed into the body near the cancer cells
  • Chemotherapy - the use of drugs to kill cancer cells. Chemotherapy may be given in many forms including: pill, injection, or by catheter. The drugs enter the bloodstream and travel through the body killing mostly cancer cells, but also some healthy cells. Chemotherapy is generally reserved for only certain types of sarcomas, such as those found in children (osteosarcoma) or when the sarcoma has spread to other parts of the body (metastatic disease).





Risk factors for Soft Tissue Sarcoma:
Symptoms - Environment  History of herbicide exposure
 A report by a 16-member committee of the National Academy of Science's Institute of Medicine found soft-tissue sarcoma associated with exposure to herbicides. Some studies have shown significant increases in soft tissue sarcoma with phenoxy herbicides.

Soft Tissue Sarcoma suggests the following may be present:
Tumors, Malignant  Cancer, General

Preventive measures against Soft Tissue Sarcoma:
Detoxification  Sauna Detoxification Program
 Sauna detoxification programs rid the body of stored toxins known to increase the risk of sarcoma.


KEY
Weak or unproven link
Strong or generally accepted link
May do some good


GLOSSARY

Abdomen (Abdominal)
That part of the body between the chest and the hips that contains the stomach, intestines, liver, bladder, pancreas and other organs.

Adrenal (Adrenal Gland, Adrenal Glands, Adrenals)
The adrenal glands sit on top of each kidney and consist of an outer cortex and an inner medulla. Of the 50 or so hormones the adrenals make, only cortisone and adrenaline are recognized by most people. Some of these hormones must be produced to preserve life, while others help resist stress. Other hormones from the adrenals control normal energy output (along with the thyroid) and govern the breakdown of stored energy into quick energy sources. The medulla produces epinephrine and norepinephrine, which are specifically designed to help the body deal with stressful situations. The adrenals control the body's potassium/sodium balance, which is vital for energy production. They also produce sex hormones in minute amounts, which is important for later years when the gonads drop or cease their production.

Alveolar Soft-Part Sarcoma
A rare cancer that mostly affects young adults. The legs are the most common location of these tumors.

Angiosarcomas (Angiosarcoma, Hemangiosarcoma, Hemangiosarcomas, Lymphangiosarcoma, Lymphangiosarcomas)
Malignant tumors that can develop either from blood vessels (hemangiosarcomas) or from lymph vessels (lymphangiosarcomas). These tumors can sometimes develop in a part of the body that has been exposed to radiation. These are sometimes seen in the breast after radiation therapy for breast cancer or in the arm on the same side as a breast that has been irradiated or removed by mastectomy.

Axillary
Pertaining to the region of the armpits.

Benign
Literally: innocent; not malignant. Often used to refer to cells that are not cancerous; they tend to grow slowly and don't spread (metastasize) like cancer tumors do.

Biopsy
Removal of a sample of tissue from a living being for diagnosis. A pathologist later uses a microscope to look for certain features, such as cancer cells, in the sample. A fine-needle aspiration biopsy involves inserting a thin needle to remove a small amount of tissue, sometimes using CT or ultrasound to guide the needle. A core biopsy involves obtaining a sample of tissue with a thick needle or by inserting a thin, lighted tube (laparoscope) into a small incision in the abdomen. Another biopsy method is to remove tissue during an operation.

Cancer
Refers to the various types of malignant neoplasms that contain cells growing out of control and invading adjacent tissues, which may metastasize to distant tissues.

Chemotherapy
A treatment of disease by any chemicals. Used most often to refer to the chemical treatments used to combat cancer cells. Chemotherapy is usually given in cycles: a treatment period followed by a recovery period, then another treatment period, and so on. Most anticancer drugs are given by injection into a blood vessel (IV); some are given by mouth. Chemotherapy is a systemic therapy, meaning that the drugs enter the bloodstream and travel throughout the body. Usually, a patient has chemotherapy as an outpatient (at the hospital, at the doctor's office, or at home). However, depending on which drugs are given and the patient's general health, a short hospital stay may be needed.

Clear Cell Sarcoma
A rare cancer that often develops in tendons and related tissues. Under the microscope, it shares some features with malignant melanoma, a type of cancer that develops from pigment-producing skin cells. How cancers with these features develop in parts of the body far from the skin is not known.

Constipation (Constipated)
Difficult, incomplete, or infrequent evacuation of dry, hardened feces from the bowels.

Dermatofibrosarcoma Protuberans (DFSP)
A low-grade cancer of the fibrous tissue beneath the skin, usually in the trunk or limbs. It invades nearby tissues but rarely metastasizes.

Desmoid Tumor (Aggressive Fibromatosis, Desmoid Tumors)
A fibrous tissue tumor with features in between fibrosarcoma and benign tumors such as fibromas and superficial fibromatosis. Desmoids do not metastasize, but they can invade nearby tissues and are sometimes fatal.

Desmoplastic Small Cell Tumor
A rare sarcoma of adolescents and young adults, found most often in the abdomen. Its name means that it is formed by small, round cancer cells surrounded by scar-like tissue.

Epithelioid Sarcoma
A sarcoma that most often develops in tissues under the skin of the hands, forearms, feet, or lower legs. Adolescents and young adults are often affected.

Fibromas (Elastofibroma, Elastofibromas, Fibroma, Fibrous Histiocytoma, Fibrous Histiocytomas, Superficial Fibromatosi, Superficial Fibromatosis)
Benign tumors of the fibrous tissue (tendons and ligaments).

Fibrosarcoma (Fibrosarcomas)
Cancer of fibrous tissue. It usually affects the legs, arms, or trunk. It is most common between the ages of 30 and 55.

Fibrous Tissue
The tissue that forms tendons and ligaments.

Glomus tumors (Glomus tumor)
Benign perivascular (around a blood vessel) tumors. They usually are found under the skin of the fingers.

Hemangioendotheliomas (Hemangioendothelioma)
Blood vessel tumors that are less aggressive than hemangiosarcomas but still considered a low-grade cancer. It usually invades nearby tissues and sometimes can spread to distant parts of the body (metastasize). It may develop in soft tissues or in internal organs, such as the liver or lungs.

Hemangiomas (Hemangioma)
Benign tumors of blood vessels. They are rather common, are often present at birth, and can affect the skin or internal organs. They sometimes disappear without treatment.

Hemangiopericytomas (Hemangiopericytoma)
Sarcomas of the perivascular (around a blood vessel) tissue. It most often develops in the legs, pelvis, and retroperitoneum (the back of the abdominal cavity). It is most common in adults.

Immune System (Immune Response, Immunity)
A complex that protects the body from disease organisms and other foreign bodies. The system includes the humoral immune response and the cell-mediated response. The immune system also protects the body from invasion by making local barriers and inflammation. The process may involve acquired immunity (the ability to learn and remember a specific infectious agent), or innate immunity (the genetically programmed system of responses that attack, digest, remove, and initiate inflammation and tissue healing).

Kaposi's Sarcoma
A cancer formed by cells similar to those lining blood or lymph vessels. In the past, Kaposi's sarcoma was an uncommon cancer mostly seen in older people with no apparent immune system problems. It is most common in people with human immunodeficiency virus (HIV) infection and the acquired immunodeficiency syndrome (AIDS), but it can also develop in organ transplant patients who are taking medication to suppress their immune system. It is probably related to infection with a virus called human herpesvirus-8 (HHV-8).

Leiomyomas (Fibroids, Fibromyoma, Leiomyoma, Myofibroma)
Benign tumors of the involuntary muscle. An encapsulated tumor made up of disorganized and irregular connective tissue. The most common of these is the "fibroid" tumor that develops in many women. It is really a leiomyoma of the uterus.

Leiomyosarcomas (Leiomyosarcoma)
Malignant tumors of involuntary muscle tissue. They can grow almost anywhere in the body but are most often found in the retroperitoneum and the internal organs where leiomyomas also arise. Less often, they develop in the deep soft tissues of the legs or arms.

Lipomas (Hibernoma, Hibernomas, Lipoblastoma, Lipoblastomas, Lipoma)
Very common benign tumors that are made of normal fat cells. They feel like soft, moveable lumps underneath the skin and do not have any malignant potential. Can be easily surgically removed. Most are found under the skin, but they can develop anywhere in the body. Lipoblastomas and hibernomas, like lipomas, are also benign fat tissue tumors.

Liposarcomas (Liposarcoma)
Malignant tumors of fat tissue. They can develop anywhere in the body, but they most often develop in the thigh and inside the back of the abdomen. This occurs mostly in adults between 50 and 65 years old. Some liposarcomas can be very slow growing, whereas others can grow quickly.

Liver (Hepatic)
The largest and one of the most complex organs of the body, the liver is responsible for much of the metabolism of fats, proteins and carbohydrates. It is the site of much of the body's detoxification. It is connected very closely with digestion and the regulation of blood sugar, among many other functions. Found behind the ribs on the right side of the abdomen, it has many important functions such as removing harmful material from the blood, making enzymes and bile that help digest food, and converting food into substances needed for life and growth. Hepatic: Pertaining to the liver.

Lung (Lungs, Pulmonary)
Organ of the body, located in the chest cavity which is designed to bring oxygen from the air into the blood stream, while also expelling carbon dioxide and other waste gases out of the body. Pulmonary: Related to the lungs.

Lymph
A clear fluid that flows through lymph vessels and is collected from the tissues throughout the body. Its function is to nourish tissue cells and return waste matter to the bloodstream. The lymph system eventually connects with and adds to venous circulation.

Lymph Nodes (Axillary Nodes, Inguinal Nodes, Lymph Gland, Lymph Glands, Lymph Node, Lymphatic)
Small, bean-shaped nodes at various points throughout the body that function to filter the lymph fluid and attempt to destroy the microorganisms and abnormal cells which collect there. The most common locations are the neck (both sides and front), armpit (axillary) and groin (inguinal), but also under the jaw and behind the ears. Swollen or painful lymph nodes generally result from localized or systemic infection, abscess formation, or malignancy. Other causes of enlarged lymph nodes are extremely rare. Physical examination for lymph nodes includes pressing on them to check for size, texture, warmth, tenderness and mobility. Most lymph nodes can not be felt until they become swollen, and then will only be tender when pressed or massaged. A lymph node that is painful even without touching indicates greater swelling. Lymph nodes can usually be distinguished from other growths because they generally feel small, smooth, round or oval-shaped and somewhat mobile when attempts are made to push them sideways. Because less fat covers the lymph nodes in children, they are easier to feel, even when they are not busy filtering germs or making antibodies. Childrens' nodes enlarge faster, get bigger in response to an infection and stay swollen longer than an adult's. Also known (incorrectly) as lymph glands.

Lymphangiomas (Lymphangioma)
Benign lymph vessel tumors that are usually present at birth.

Lymphoma (Lymphomas)
Any tumor of the lymphatic tissues.

Malignant (Cancerous)
Dangerous. Mainly used to describe a cancerous growth -- when used this way, it means the growth is cancerous and predisposed to spreading.

Malignant Fibrous Histiocytoma (MFH)
The most common malignant soft tissue tumor found in the arms or legs. Less often, it can develop inside the back of the abdomen. This sarcoma is most common in older adults.

Malignant Mesenchymoma
A rare type of sarcoma that contains some areas showing features of fibrosarcoma and other areas with features of at least two other types of sarcoma.

Metastasize (Metastases, Metastasized, Metastasizes, Metastasizing, Metastatic)
Usually in reference to cancer: to spread, especially destructively.

Myxoma (Myxomas)
A benign tumor that usually is located in muscles but does not develop from muscle cells. The cells of a myxoma produce mucous-like material, a feature that distinguishes this tumor.

Neurofibromas (Neurofibroma, Neuroma, Neuromas, Schwannoma, Schwannomas)
Benign tumors of nerves.

Neurofibrosarcomas (Malignant Schwannoma, Malignant Schwannomas, Neurofibrosarcoma, Neurogenic Sarcoma, Neurogenic Sarcomas)
Malignant tumors of the cells that surround a nerve. A new term for these is malignant peripheral nerve sheath tumors.

Nodular Tenosynovitis
A benign tumor of joint tissue. It is most common in the hands and is more common in women than in men.

Pelvis
The lower part of the abdomen between the hip bones. Organs in a woman's pelvis include the uterus, vagina, ovaries, fallopian tubes, bladder and rectum.

Perivascular
Describing the area round the blood vessels.

Polyp (Polyps)
A usually nonmalignant growth or tumor protruding from the mucous lining of an organ such as the nose, bladder or intestine, often causing obstruction.

Radiation Therapy (Radiotherapy)
The use of high-energy rays to kill cancer cells. Depending on the stage of the disease, treatment with radiation may be given alone or with chemotherapy. Radiation therapy is local therapy; it affects cancer cells only in the treated area. External radiation does not cause the body to become radioactive. Most often, treatment is given on an outpatient basis in a hospital or clinic.

Rhabdomyomas (Rhabdomyoma)
Benign tumors of skeletal muscle (the muscle that is attached to bone and helps us to move).

Rhabdomyosarcomas (Rhabdomyosarcoma)
Malignant tumors of skeletal muscle. These tumors commonly grow in the arms or legs, but they can also begin in the head and neck area and in reproductive and urinary organs such as the vagina or bladder. Children are affected much more often than adults.

Smooth Muscle (Involuntary Muscle)
The human body contains 2 types of muscle - smooth muscle and striated (skeletal) muscle. Smooth muscle is found in our internal organs such as stomach, intestines, blood vessels, or uterus (womb) and causes them to contract. These muscles are involuntary - that is, we don't control their movement.

Staging
Although grading and the identification of cancer cell types help determine a patient's prognosis, most doctors believe that the cancer's stage is a better indicator of a patient's survivability. The grade of a cancer cell is an assessment of its appearance compared to that of a normal, healthy cell. Grading is done on a scale (such as 1 to 4) with grade 1 cells differing little from normal cells, typically spreading slowly, and having a good prognosis for treatment. The highest grade cells look extremely different and indicate an aggressive cancer with poor prognosis. Staging allows a physician to gauge the size and location of tumors by using information gathered from imaging studies such as CT scans and MRIs, and from pathology tests and physical examinations. Staging factors that influence a patient's prognosis are:



Striated Muscle (Skeletal Muscle, Voluntary Muscle)
The human body contains 2 types of muscle - smooth muscle and striated (skeletal) muscle. Striated muscle (striped when seen under the microscope) is the muscle that allows us to move our arms and legs and other body parts on purpose - that is, voluntary movement.

Syndrome
A medical condition characterized by a collection of related symptoms (what the patient feels) and signs (what a doctor can observe or measure).

Synovial Sarcoma
A malignant tumor of the tissue around joints. The most common location is the knee. It tends to occur in young adults.

Synovium (Synovial)
The tough tissue that surrounds all of our joints, and which produces the fluid that lubricates the joint surfaces so that they move smoothly.

Uterus (Uterine)
The part of the female reproductive system specialized to allow the implantation, growth and nourishment of a fetus during pregnancy.

X-rays (X-ray)
High-energy radiation used to take pictures of areas inside the body.




Last updated: Apr 13, 2008


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