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Childhood Soft Tissue Sarcoma Treatment

General Information About Childhood Soft Tissue Sarcoma

Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in soft tissues of the body.

Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following:

Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen).

There are many different types of soft tissue sarcomas.

The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed.

Rhabdomyosarcoma is the most common type of childhood soft tissue sarcoma. It begins in muscles that surround bone. Rhabdomyosarcoma is not discussed in this summary. (See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.) This summary is about the other types of soft tissue sarcoma:

Fibrous (connective) tissue tumors

Fibrohistiocytic tumors

Fat tissue tumors

Smooth muscle tumors

Peripheral nervous system tumors

Bone and cartilage tumors

PEComas

Tumors with more than one type of tissue

Tumors of unknown origin (the place where the tumor first formed is not known)

Blood and lymph vessel tumors

Besides rhabdomyosarcomas, the most common soft tissue sarcomas in children are in joint tissue, connective tissue, and nerve tissue.

Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment, and may have a better outcome than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information on treatment in adults.)

Having certain diseases and inherited disorders can increase the risk of developing childhood soft tissue sarcoma.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:

Other risk factors include the following:

The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body.

A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, or the trunk. There may be no other symptoms at first. As the sarcoma grows larger and presses on nearby organs, nerves, muscles, or blood vessels, symptoms may occur, including pain or weakness.

Other conditions may cause the same symptoms that soft tissue sarcomas do. A doctor should be consulted if any of these problems occur.

Diagnostic tests and a biopsy are used to detect (find) and diagnose childhood soft tissue sarcoma.

The following tests and procedures may be used:

If these tests show there may be a soft tissue sarcoma, a biopsy is done. One of the following types of biopsies may be used:

In order to plan the best treatment, a large sample of tissue may be removed during the biopsy to find out the type of soft tissue sarcoma and do laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have a tumor. A pathologist views the tissue under a microscope to look for cancer cells and to find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade and mid-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, patients should ask to have the tissue sample checked by a pathologist who has experience in diagnosing soft tissue sarcoma.

One or more of the following laboratory tests may be done to study the tissue samples:

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following: