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Sarcoma

Anatomical facts:

The connective tissues of the body have two components, bones and soft tissues. A majority of the bones are hollow from within and contain a substance known as marrow (which is composed of blood forming cells and fat cells). Located at the terminal point of each bone is a region of cartilage which provides a cushioning surface between bones. Along with ligaments and various other tissues, this cartilage forms the joints found between all bones. Soft tissue is a name given to a group of tissues comprised of blood vessels, muscles, fat, cartilage, nerves, tendons and deep skin tissues.

What is sarcoma?

Sarcoma is the name given to a group of cancers which initiate from the connective tissues of the body. According to an estimate given by the American Cancer Society, there are almost 9,200 cases of soft tissue sarcomas which are diagnosed every single year, out of which about 2300 cases are primary bone sarcomas. Sarcomas can affect adults as well as children.

Types of sarcomas:

There are more than one hundred different kinds of sarcoma cancers found in the world. It is nearly impossible to give the individual survival statistics of each and every type of sarcoma. Sarcomas are named based on the names of the tissues that they grow from. For instance:

  • Fibrosarcoma is the sarcoma which originates from the fibrous tissues of the body such as the ligaments and tendons
  • Liposarcoma is the name given to the sarcoma which arises in the fatty cells
  • Chondrosarcoma originates from the different cartilages of the body and
  • Leiomyosarcoma is the type of cancer which arises from the smooth muscle tissues of the body
  • Osteosarcoma is the cancer which is primary in bones.

It is important for patients to understand that sarcoma is a primary cancer, and not a tumor which has metastasized from another location of the body to the bone or soft tissues. It has been noted that most of the tumors that are found occurring in bones are of the secondary type and not primary tumors.

Soft tissue sarcoma:

Soft tissue sarcomas are malignant cancerous tumors that start from the soft tissues of the body. Soft tissues are the tissues that support, connect and surround the other structures of the body[1]. Many different types of sarcomas can actually occur from the soft tissues of the body and can spread to other parts of the body.

Occurrence:

Nearly 40% of all the sarcomas diagnosed all over the world are found occurring in the legs or above the knees in both adults and children. Almost 15% of sarcoma cases have been observed occurring in the arms or the hands and 15% are seen in the neck and head region of the body. About 30% of the sarcomas occur in the hips, shoulders, abdomen or the chest. It has been seen that, in most cases, sarcoma is not associated with a particular risk factor or causative agent which may have contributed towards the development or progress of cancer in an individual.

However, some of the common risk factors which are linked with the occurrence of sarcoma include exposure to ionizing radiation as well as various chemicals that the person may have contacted. There are a number of medical conditions which may increase the risk of a person to develop sarcomas. One of the most prominent of these genetic conditions which can place a person at a risk of developing sarcoma is neurofibromatosis.

Treatment of sarcomas:

The most effective form of treatment for cure of any kind of sarcoma is surgery. With the help of surgical procedures, the tumor, as well as a margin of about an inch of normal tissue which surrounds the tumor, can be removed safely without damaging or harming any other part of the body in the process. The surgery is rather difficult if the tumor is located in the pelvic region or the abdomen because there are a number of vital organs in those areas. In cases where the sarcoma has spread and has grave severity, an amputation may be necessary in order to effectively remove the cancerous lesion from the body. Many of the sarcomas which are found occurring in the legs or the arms can be treated with the help of a surgery known as “limb sparing surgery”, which is then followed by radiation therapy.

It must be noted that if the sarcoma has metastasized, or spread from one part of the body to another, surgical procedures may not be entirely effective in providing a complete cure against the disease. In case there is only one metastasis of the cancer either in the brain or in the lung, surgery can be effective for complete cure. Here, the patient may have a good rate of survival because the metastasis is removed from the body along with the primary cancerous lesion. As part of the adjuvant therapy following surgery, chemotherapy and radiation therapy can be used. There is a type of sarcoma, known as gastrointestinal stromal tumor, which can be cured with the help of a drug known as imatinib mesylate.

[1] www.mayoclinic.com/health/soft-tissue-sarcoma/DS00601

The NW Sarcoma Foundation. Access at: www.nwsarcoma.org The mission of this organization is to provide education and support to patients and families living with this cancer. The site includes explanations of sarcoma, its biology and pathology, and treatment options, with color illustrations that help understand the complex anatomy of the skeletal system. The section on support provides links to many organizations and resources that may be of help.

American Cancer Society. Learn about Bone Cancer and Learn about Sarcoma Adult Soft Tissue Cancer. Access at: www.cancer.org and select from the menu under “Choose a Cancer Topic.” Detailed guides about sarcoma can be printed by section or as Adobe Acrobat (.pdf) files.

National Cancer Institute. Bone Cancer and Soft Tissue Sarcoma. Access at: www.cancer.gov and click on “Types of Cancer.” The top page of the section links to patient versions of PDQ@ statements, clinical trial information, and other NCI publications on this topic.

People Living with Cancer. Sarcoma. Access at: www.plwc.org and click on “Cancer Type.” The PLWC oncologist-approved sarcoma guide can be printed in its entirety or by subtopic.

Sarcoma Alliance. Access at: www.sarcomaalliance.org  The site of this patient-support organization offers a comprehensive list of links to other Websites and articles about soft tissue and bone sarcomas. The section “The First Thing You Should Know” offers advice to newly diagnosed patients on how to get quality medical care and support. This site also includes a list of sarcoma centers, specialists, and clinical trial registries.

National Cancer Institute. Soft Tissue Sarcomas: Questions and Answers and Bone Cancer: Questions and Answers. These are short publications that cover risk factors, diagnosis, and
treatment of soft tissue and bone sarcomas. Access at: www.cancer.gov

This article was originally published on 7/12/2014 and last revision and update of it was 9/14/2015.