Sarcomas of Specific Organs: Overview

Approved by the Cancer.Net Editorial Board, 07/2014

ON THIS PAGE: You will find some basic information about these diseases and the parts of the body they may affect. This is the first page of Cancer.Net’s Guide to Sarcomas of Specific Organs. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About sarcoma

Soft tissue sarcoma is cancer that develops in the tissues that support and connect the body. A sarcoma can occur in fat tissue, muscles, nerves, tendons, joints, blood vessels, or lymph vessels. A sarcoma begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread.

When a sarcoma is small, it usually does not cause problems and may go unnoticed or appear harmless. Most people with a small soft tissue sarcoma can be treated successfully. However, if the sarcoma grows, it can interfere with the body's normal activities, and it can spread to other parts of the body. This makes it more challenging to treat successfully.

Sarcoma can begin in any part of the body. About 50% start in an arm or leg, 40% start in the trunk or abdomen, and 10% start in the head or neck. Sarcoma is uncommon, accounting for about 1% of all cancers.

About sarcomas unique to particular organs

Because there are several different types of soft tissue sarcoma, it is considered a family of related diseases, rather than a single, specific disease. Some of the sarcomas unique to particular organs are listed below.

Body Part

Unique Sarcoma(s)

Aorta, pulmonary artery, pulmonary vein (large blood vessels coming out of the heart)

Intimal sarcoma

Brain and its coverings, called meninges

Solitary fibrous tumor


Phyllodes tumor, angiosarcoma


Angiosarcoma, rhabdomyosarcoma; benign myxomas are more common


Embryonal sarcoma, epithelioid hemangioendothelioma

Lymph nodes or other lymphatic tissue

Lymphangioleiomyomatosis, perivascular epithelial cell tumor (PEComa), true histiocytic sarcoma, follicular dendritic cell tumor, interdigitating cell tumor

Pleura, the lining around the lungs

Epithelioid hemangioendothelioma, solitary fibrous tumor, synovial sarcoma


Rhabdomyosarcoma, prostatic stromal sarcoma


Endometrial stromal sarcoma, undifferentiated endometrial sarcoma, adenosarcoma, PEComa

Each of these sarcomas has different characteristics. Some of the sarcomas listed above are relatively slow growing, while others can grow quite quickly. For example, phyllodes tumors can grow quickly but do not travel to lymph nodes like most common breast cancers.

Some of the rare sarcomas specific to particular organs listed above are discussed in this section. You can learn more about soft tissue sarcoma in a separate section on Cancer.Net.

Looking for More of an Overview?

If you would like additional introductory information, view a short video led by an ASCO expert in sarcoma that provides basic information and areas of research. Please note this link will take you to another section of Cancer.Net.

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