Soft Tissue Sarcoma and Connective Tissue Tumors

Soft tissue sarcoma is a rare type of cancer that occurs in the connective tissues in the body, affecting approximately 13,000 patients per year in the United States. Since soft tissue sarcoma is rare, it is best managed by oncologists with expertise and a focus on these tumors.

About Soft Tissue Sarcoma and Connective Tissue Tumors

Soft tissue sarcomas are a group of cancerous tumors that can occur in any part of the body, including the head and neck, arms and legs, trunk, or the retroperitoneum (back of the abdominal cavity). Oncologists who treat these tumors also frequently manage other types of connective tissue tumors, many of which are not cancerous.

Types of Soft Tissue Sarcomas

Soft tissue sarcomas are found in the soft tissues that connect, support, and surround other body structures. This includes muscle, fat, blood vessels, nerves, tendons, and the lining of your joints. There are more than 50 different types of soft tissue sarcomas.

Examples of soft tissue sarcomas:

• Angiosarcoma
• Dermatofibrosarcoma protuberans (DFSP)
• Fibrosarcoma
• Gastrointestinal stromal tumor (GIST)
• Leiomyosarcoma
• Liposarcoma
• Myxofibrosarcoma
• Malignant peripheral nerve sheath tumors
• Rhabdomyosarcoma
• Synovial sarcoma
• Undifferentiated pleomorphic sarcoma

Tumors that have “sarcoma” in the name are malignant, meaning they are cancerous and can spread to other parts of the body.

Types of Connective Tissue Tumors

Connective tissue tumors are also found in the soft tissues that connect, support, and surround other body structures. There are many types of soft tissue tumors, some of which are benign, meaning they are not cancerous and do not spread to other parts of the body.

Examples of connective tissue tumors with uncertain malignant potential:

• Atypical fibroxanthoma
• Solitary fibrous tumors

Examples of benign connective tissue tumors:

• Desmoid tumor
• Leiomyoma
• Lipoma
• Neurofibroma
• Gangioneuroma
• Schwannoma

Symptoms of Soft Tissue Sarcomas and Connective Tissue Tumors

Soft tissue sarcoma and connective tissue tumor symptoms depend on the location of the tumor.

In the extremities:

• Palpable mass (a mass you can feel)
• Swelling of the limb

In the retroperitoneum:

• Bloating
• Constipation
• Nausea

Risk Factors for Soft Tissue Sarcoma and Connective Tissue Tumors

Most soft tissue sarcoma and connective tissue tumors occur without a known risk factor or genetic syndrome. However, in some cases, the tumor can be traced back to certain risk factors or genes.

Common risk factors and genetic syndromes related to soft tissue sarcoma and connective tissue tumors:

• Prior high dose radiation exposure
• Lymphedema
• Genetic syndromes:
  • Gardner syndrome
  • Li-Fraumeni syndrome
  • Von Recklinghausen disease
  • Inherited retinoblastoma
  • Werner syndrome
  • Gorlin syndrome
  • Tuberous sclerosis

Treating Soft Tissue Sarcoma and Connective Tissue Tumors at UT Medicine 

Treatment requires accurate diagnosis and a care plan tailored to the specific type of tumor, the tumor’s location, and the overall needs of the patient. The different behavior of different tumor types helps drive decisions about treatment, which may include combinations of surgery, radiation therapy, and chemotherapy.