Sarcomas of Specific Organs: Introduction

Approved by the Cancer.Net Editorial Board, 06/2020

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. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

About sarcoma

Soft-tissue sarcoma (STS) is cancer that develops in the tissues that support and connect the body. Sarcomas can occur in fat tissue, muscles, nerves, tendons, joints, blood vessels, or lymph vessels. A sarcoma begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but 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 STS 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. Sarcoma that has spread is more challenging to treat successfully.

Sarcoma can begin in any part of the body. About 60% start in an arm or leg, 30% start in the trunk or abdomen, and 10% start in the head or neck. Sarcoma is uncommon and accounts for about 1% of all cancers.

About sarcomas found in specific organs

Because there are several different types of STS, it is considered a family of related diseases and not a single, specific disease. Sarcomas that are found in a specific organ have different names and are different from other sarcomas. Some of these organ-specific sarcomas are listed below.

Body Part

Specific 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. Others can grow quite quickly. For example, phyllodes tumors can grow quickly but, unlike most common breast cancers, only very rarely spread to lymph nodes.

Some of the rare sarcomas specific to particular organs listed above are discussed in this section. You can learn more about STS in a separate section of this same website.

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If you would more of an introduction, explore these related items. Please note that these links will take you to another section of Cancer.Net:

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with sarcoma and general survival rates. Use the menu to choose a different section to read in this guide.