Childen's Hospital Boston 300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
X
My Child Has:
Soft Tissue Sarcomas
Programs that treat this condition
Bone and Soft Tissue Tumor Program  
What are soft tissue sarcomas?
Soft tissue sarcomas are cancerous tumors that begin in the tissues that connect, support or surround organs and other body structures. Soft tissue includes fat, muscles, tendons (bands of fiber that connect bone to muscle) nerves, synovial (joint) tissue, blood vessels, and other fibrous tissue.

As you read further below, you will find general information about soft tissue sarcomas. If you would like to view summary information about cancer first, see the cancer overview.

Soft tissue sarcomas can develop almost anywhere in the body. About half occur in the arms, legs, hands or feet. The rest develop in the chest, back, abdomen, shoulders, hips, and head and neck area. Soft tissue sarcoma cells can invade surrounding tissue and metasticize (spread) to other organs of the body.

A relatively uncommon cancer, soft tissue sarcomas account for less than 1 percent of all new cancer cases each year. In the United States, approximately 900 children and adolescents are diagnosed with soft tissue sarcomas each year.

What causes soft tissue sarcomas?
The exact cause of soft tissue sarcomas is not entirely understood, however, studies have indicated that genetic alterations may play a role. Researchers have studied a small number of families that contain several members of one generation who have developed soft tissue sarcomas. In addition, limited studies have shown a possible link between soft tissue sarcomas and the development of other types of cancer.

Certain inherited diseases are also associated with an increased risk of developing soft tissue sarcomas. These include people with Li-Fraumeni syndrome (which involves alterations in the p53 gene) or neurofibromatosis (which involves alterations in the NF1 gene). For some soft tissue tumors, there seems to an association with an Epstein-Barr virus infection.

What are the different types of soft tissue sarcoma?
There are many different types of soft tissue sarcoma, the most common of which is rhabdomyosarcoma, a tumor of skeletal muscles. See the rhabdomyosarcoma section for more specific information on this type of soft tissue tumor.

Another type of soft tissue tumor is Desmoid tumor, also called aggressive fibromytosis, which affects the fibrous tissue that makes up tendons and ligaments. This tumor is considered to be benign because it does not spread. See the Desmoid tumor section for more specific information on this type of soft tissue tumor.

Other soft tissue sarcomas experienced by children include:

  • infantile fibrosarcoma - a tumor that develops in the tissue that forms tendons and ligaments
  • leiomyosarcoma - a tumor that develops in involuntary muscle tissue, usually found in the abdomen, bowels, uterus, and blood vessels
  • liposarcoma - a tumor that develops in fat tissue, most often in the abdominal cavity
  • malignant fibrous histiocytoma - a tumor that begins in the fibrous tissue in the legs
  • peripheral nerve sheath tumor - a tumor that develops in the cells that surround nerves
  • synovial cell sarcoma - a tumor that develops in the tissue around joints, usually in the knee
What are the symptoms of soft tissue sarcomas?
Because soft tissue sarcomas affect tissue that is elastic and easily moved, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue. The following are the most common symptoms of soft tissue sarcomas. However, each child may experience symptoms differently. The symptoms of soft tissue sarcomas vary greatly depending on size, location, and spread of the tumor. Symptoms may include:
  • a painless swelling or lump
  • pain or soreness caused by compressed nerves or muscles
  • limping or other difficulty using the legs, feet, arms or hands
The symptoms of soft tissue sarcomas may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
How are soft tissue sarcomas diagnosed?
In addition to a complete medical and physical examination, the most conclusive diagnostic procedure for soft tissue sarcomas is a biopsy, a tissue sample from the tumor taken through a simple surgical procedure.

Diagnosis involves classifying the tumor, which is important in determining treatment options. The process helps determine whether cancer has spread, and, if so, how far. There are various systems used for soft tissue sarcomas. Always consult your child's physician for more information. One method evaluates the disease after surgery and classifies it by one of the four following groups:

  • group I - tumor completely removed with no evidence of cancer in surrounding tissue; no evidence that the tumor has metastasized
  • group II - tumor removed with some evidence of cancer in surrounding tissue; no evidence that the tumor has metastasized
  • group III -tumor partially removed with evidence of cancer in surrounding tissue; no evidence that the tumor has metastasized
  • group IV - any tumor combined with evidence that the tumor has metastasized (spread)
What are the treatments for soft tissue sarcomas?
Specific treatment for soft tissue sarcomas will be determined by your child's physician based on:
  • your child's age, overall health, and medical history
  • extent of the disease
  • your child's tolerance for specific medications, procedures, or therapies
  • how your child's doctor expects the disease may progress
  • your opinion or preference
Treatment may include:
  • surgery - biopsy and surgical removal of tumors, performed by a surgeon. Depending on the size and location of the tumor, it is sometime necessary to remove all or part of a limb. In most cases, however, limb-sparing surgery is used to avoid amputation. Through limb-sparing surgery, as much of the tumor as possible is removed. Radiation therapy and/or chemotherapy are given either before surgery to shrink the tumor, or after surgery to kill remaining cancer cells.
  • radiation therapy - using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors
  • chemotherapy - a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation or surgery. Often, a combination of chemotherapy drugs is used to fight a specific cancer. Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat. While chemotherapy can be quite effective in treating certain cancers, the agents do not differentiate normal healthy cells from cancer cells. Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

    • as a pill to swallow
    • as an injection into the muscle or fat tissue
    • intravenously (directly to the bloodstream; also called IV)
    • intrathecally - chemotherapy given directly into the spinal column with a needle
  • supportive care - any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment
  • 7 continuous follow-up care - a schedule of follow-up care determined by your child's physician and other members of your care team to monitor ongoing response to treatment and possible late effects of treatment
Treatment options will vary greatly, depending on your child's individual situation. Your child's physician and other members of your care team will discuss these options with you in-depth.
What is the long-term outlook for patients with soft tissue sarcomas?
Prognosis greatly depends on:
  • the extent of the disease
  • the size and location of the tumor
  • the size and location of the tumor
  • a presence or absence of metastasis
  • the tumor's response to therapy
  • the age and overall health of your child
  • your child's tolerance of specific medications, procedures, or therapies
  • new developments in treatment
As with any cancer, prognosis and long-term survival can vary greatly from individual to individual. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with soft tissue sarcomas. Side effects of radiation and chemotherapy, as well as recurrence of the disease, can occur in survivors of soft tissue sarcomas.
What is the latest research on soft tissue sarcomas?
Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians better understand and treat soft tissue sarcomas.

Other types of treatment currently being studied include:

  • angiogenesis inhibitors - substances that may be able to prevent the growth of tumors by blocking the formation of new blood vessels that feed the tumors
  • biological therapies - a wide range of substances that may be able to involve the body's own immune system to fight cancer or lessen harmful side effects of some treatments
X
X The information on this website should not be taken as medical advice,
which can only be given to you by your personal health care professional.
X
X Copyright © Children's Hospital Boston. All rights reserved.