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Sarcoma

This section has been reviewed and approved by the Cancer.Net Editorial Board, 8/09

Overview

Overview


Cancer begins when cells begin to change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

Soft tissue sarcomas (STS) are a group of cancers that develop in the tissues that support and connect the body. STS can occur almost anywhere in the body, and the sarcoma cells resemble the cells that hold the body together, including fat cells, muscle, nerves, tendons, joints, blood vessels, or lymph vessels. When an STS is small, it can go unnoticed or is ignored, since it does not usually cause problems at this stage. As an STS grows, it can interfere with the body's normal activities.

This section covers sarcoma of the soft tissues. For information on a sarcoma that starts in a bone, read the Guide to Bone Cancer. For information on a type of STS called gastrointestinal stromal tumor (GIST) that affects the gastrointestinal tract, read the Guide to GIST.

Sarcomas can appear in any part of the body. About 60% occur in the arms or legs, 30% occur in the trunk or abdomen, and 10% occur in the head or neck. Sarcoma is rare in adults, accounting for about 1% of all adult cancers. However, sarcoma in general, represent about 15% of all cancers in children.

Because there are several different types of STS, it is probably best considered as a family of related diseases, rather than a single, specific disease. The specific types of sarcoma are frequently named according to the normal tissue cells they most closely resemble (Table 1). This is different from how most other types of caner are named by referring to any anatomical site (part of the body) where the disease started.

Table 1: Types of Sarcoma



Name of Sarcoma


Related Normal Tissue Type


Angiosarcoma


Blood or lymph vessels
Ewing’s sarcoma (peripheral primitive neuroectodermal tumor, PNET) Mesenchymal stem cells (cells that develop into connective tissue, blood vessels, and lymphatic tissue)


Fibrosarcoma


Fibrous tissue (tendons and ligaments)


Gastrointestinal stromal tumor (GIST)


Specialized neuromuscle cells of the digestive tract
Kaposi’s sarcoma Blood vessels


Leiomyosarcoma


Smooth muscle


Liposarcoma


Fat tissue
Myxofibrosarcoma (myxoid malignant fibrous histiocytoma [MFH]) Connective tissue


Malignant peripheral nerve sheath tumor (MPNST, also known as neurofibrosarcoma)


Peripheral nerve
Pleomorphic sarcoma, not otherwise specified (NOS; often referred to in the past as malignant fibrous histiocytoma, MFH) Undifferentiated connective tissue stem cells


Rhabdomyosarcoma


Skeletal muscle


Synovial sarcoma


Primitive mesenchymal stem cells


Grade is the term a pathologist (a doctor who specializes in interpreting laboratory tests and diagnosing disease) uses to describe how aggressive the sarcoma is likely to be, especially in terms of risk of spreading to another site in the body. A low-grade tumor usually has a lower risk of spreading rapidly, and it often stays in the place where it started. A high-grade tumor is more likely to spread to other places, a process known as metastasis.

Experts have identified many types and subtypes of sarcomas. Pathologists are now trying to find new ways to quickly determine a tumor's subtype, as this helps determine treatment. Examination of a tumor's abnormal genetic code may help determine its characteristics and predict which treatments will be most effective. For at least two types of sarcoma, GIST and dermatofibrosarcoma protuberans (DFSP), major advances have been made in a type of treatment called targeted therapy (drug treatments targeted to specific genetic abnormalities in the sarcoma cells).

Statistics

In 2009, approximately 10,660 people (5,780 males and 4,880 females) will be diagnosed with soft tissue sarcoma in the United States. An estimated 3,820 adults and children (1,960 males and 1,860 females) are expected to die of the disease this year.

The overall five-year relative survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases), combining all stages and types of sarcoma, is approximately 50%. If the sarcoma is diagnosed at an early stage and hasn’t spread from the original location of the tumor, the five-year survival rate is 83%. The five-year survival rate for cancer that has spread to the lymph nodes is 54%, and the five-year survival rate is 16% if cancer has spread to other parts of the body. However, when the sarcoma is located in an arm or leg, the five-year survival rates are slightly higher for each stage.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with sarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer.

Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2009, and from data from the National Cancer Institute and Memorial Sloan-Kettering Cancer Center.

Find out more about basic cancer terms used in this section.

 
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Last Updated: November 25, 2009