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Sarcoma Definitions

A sarcoma is a highly malignant and rare type of cancer that arises in the connective tissues in the body.

There are approximately 9,800 new cases of soft tissue and bone sarcoma each year in the United States. While rare among adults (less than 2% of all adult cancers), sarcoma is one of the top five most commonly diagnosed cancers among children.

Some of the most common subtypes of sarcoma include:

Angiosarcoma - cancerous tumors originating from blood vessels.

Chondrosarcoma - cancerous tumors of cartilage.

Desmoplastic Small Round Cell Tumors (DSRCT) - Soft tissue sarcomas primarily affecting children and young adults.  The tumors usually occur as masses in the abdomen, but other areas may be affected. Not much is known about DSRCT as it is a relatively “new” cancer only first described in 1989.

Ewing's Sarcoma - (also known as Peripheral Neuroectoderman Tumor or "PNET") - This form of sarcoma generally arises from very primitive cells in the body. It is usually classified as a bone tumor, however it can also originate in soft tissues in the body.

Fibrosarcoma - Cancer that arises in the fibroblast - type cells (any cell from which connective tissue is developed) in the body. These cells form scars as well as perform other important connective functions. A sarcoma containing much connective tissue.

Gastrointestinal Stromal Tumor (GIST) - A form of cancer of the connective tissue that supports the gastrointestinal ("GI") tract. This form of sarcoma is also referred to as "GI Stromal Sarcoma."

Hemangiosarcoma - a cancerous tumor originating from blood vessels. Syn:Angiosarcoma

Kaposi's Sarcoma - also known as multiple idiopathic hemorrhagic sarcoma - originates in multiple sites in the mid - dermis (second layer of skin and extends to the epidermis (outer layer of skin). Some forms of KS look nodular or plaque - like on the skin. Lymphadenopathic form of KS is disseminated (scattered or distributed over a considerable area) and aggressive, involving lymph nodes, viscera, and occasionally the GI tract.

Leiomyosarcoma - Cancerous tumors that originate in the smooth muscle tissue. (Smooth muscle is muscle over which the brain does not have conscious control. Examples include the muscles in the walls of blood vessels, the uterus and GI tract.) Leiomyosarcoma can originate anywhere in the body but the uterus or gastrointestinal tracts are the most common sites of origination.

Liposarcoma - cancerous tumors which arise from fat tissue. They can develop anywhere in the body, however they most often originate in the retroperitoneum (lining at the back of the abdominal cavity).

Lymphangiosarcoma - cancer that develops from the endothelial lining of the lymphatics.

Malignant Fibrous Histiocytoma (MFH) - An often times controversial form of sarcoma due to its uncertain histogenesis and numerous subtypes. Still this form of sarcoma is perhaps the most common form of common soft tissue sarcomas (accounting for 20 - 24% of soft tissue sarcomas) diagnosed in late adult life.

Malignant Peripheral Nerve Sheath Tumor (MPNST) - A cancer of the cells that surround the nerves. This form of cancer is also often referred to as neurofibrosarcoma.

Neurofibrosarcoma - A cancer of the cells that surround the nerves. This form of cancer is often referred to as Malignant Peripheral Nerve Sheath Tumor (MPNST).

Osteogenic Sarcoma - A form of cancer that arises in bone. This form of sarcoma is also known as osteosarcoma.

Osteosarcoma - A form of cancer that arises in bone. This form of sarcoma is also known as osteogenic sarcoma.

Peripheral Neuroectodermal Tumor of "PNET" - (also known as Ewing's Sarcoma)this form of sarcoma generally arises from very primitive cells in the body. It is usually classified as a bone tumor, however it can also originate in soft tissues in the body.

Rhabdomyosarcoma - Cancerous tumors that originates in muscle tissue. These tumors are most common in the arms or legs, but can also develop in the head, neck, urinary, or reproductive organ areas. This is one of the most common forms of sarcoma diagnosed among children. Rhabdomyosarcoma is one of four types of malignant cardiac tumors (cancer of the heart).

Solitary Fibrous Tumor (SFT) – Rare mesenchymal tumors originating in the pleura or at virtually any site in the soft tissue. While not “technically” a form of sarcoma, it is highly recommended that treatment be administered by a sarcoma specialist. 

Stewart-Treves Syndrome - A rare, cutaneous angiosarcoma (malignant tumor of vascular origin) that can develop as a result of long-standing chronic lymphedema (swelling due to obstruction of the lymphatic system).  Most commonly, Stewart-Treves occurs as a result of lymphedema induced by radical mastectomy and/or radiation to treat breast cancer, although it can result in non-breast cancer patients who experience chronic lymphedema for other reasons.

Synovial Sarcoma - This form of sarcoma is comprised of cells that resemble the cells in joints. While synovial cells normally line the joints of the body, synovial sarcoma does not necessarily arise in any joint. The name of this particular form of sarcoma is probably a misnomer, since the cancer cells in synovial sarcoma are likely to be very different from normal joint cells, synovial sarcomas can originate in any location in the body, and often appear in young adults.


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