Thomas Jefferson UniversitySidney Kimmel Medical College

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Soft Tissue Sarcoma

Quick Facts

Soft tissue sarcomas are malignant tumors that may arise in any of the mesodermal tissues of the extremities (50%), trunk and retroperitoneum (40%), or head and neck (10%). Soft tissue sarcomas occur with greater frequency in patients with the following: Basal cell syndrome, Gardner syndrome, Li-Fraumeni syndrome (p53 mutations), Tuberous sclerosis, von Recklinghausen disease (neurofibromatosis), Werner syndrome. The prognosis for patients with adult soft tissue sarcomas depends on several factors, including the patient’s age, the size, histologic grade, and stage of the tumor. Factors associated with a poorer prognosis include Age older than 60 years, Tumors larger than 5 cm, High-grade histology.

Specialized Physicians:
Atrayee Basu Mallick, MD
Avnish Bhatia, MD
Christina Brus, MD
Andrew E. Chapman, DO, FACP
Michael J. Ramirez
Lewis J. Rose, MD, FACP
Allison Zibelli, MD, FACP

For further information contact:
Thomas Jefferson University Hospital
www.jeffersonhospital.org/cancer

Definition

A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body. The soft tissues of the body include the muscles, tendons (bands of fiber that connect muscles to bones), fat, blood vessels, lymph vessels, nerves, and tissues around joints. There are many types of soft tissue sarcoma. The cells of each type of sarcoma look different under a microscope, based on the type of soft tissue in which the cancer began.

Adult soft tissue sarcoma is a disease in which malignant (cancer) cells form in the soft tissues of the body. Adult soft tissue sarcomas can form almost anywhere in the body, but are most common in the legs, abdomen, arms, and trunk.

Stage I: is divided into stages IA and IB:

  • In stage IA, the tumor is low-grade (likely to grow and spread slowly) and 5 centimeters or smaller. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).
  • In stage IB, the tumor is low-grade (likely to grow and spread slowly) and larger than 5 centimeters. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).

Stage II:  is divided into stages IIA and IIB:

  • In stage IIA, the tumor is mid-grade (somewhat likely to grow and spread quickly) or high-grade (likely to grow and spread quickly) and 5 centimeters or smaller. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).
  • In stage IIB, the tumor is mid-grade (somewhat likely to grow and spread quickly) and larger than 5 centimeters. It may be either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue).

Stage III: In stage III, the tumor is either high-grade (likely to grow and spread quickly), larger than 5 centimeters, and either superficial (in subcutaneous tissue with no spread into connective tissue or muscle below) or deep (in the muscle and may be in connective or subcutaneous tissue); or any grade, any size, and has spread to nearby lymph nodes.

Stage IV: In stage IV, the tumor is any grade, any size, and may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body, such as the lungs.

Treatment

Treatment of stage I soft tissue sarcoma may include the following: Surgery (wide local excision or Mohs microsurgery) or Radiation therapy before and/or after surgery. If cancer is found in the head, neck, abdomen, or chest, treatment may include the following: Surgery, Radiation therapy before or after surgery, or Fast neutron radiation therapy. Treatment of stages II and III adult soft tissue sarcoma may include the following: Surgery (wide local excision), Surgery (wide local excision) with radiation therapy for large tumors, High-dose radiation therapy for tumors that cannot be removed by surgery, or radiation therapy or chemotherapy before limb-sparing surgery. Radiation therapy may also be given after surgery or a clinical trial of surgery followed by chemotherapy, for large tumors. Treatment of stage IV adult soft tissue sarcoma that involves lymph nodes may include the following: Surgery (wide local excision) with or without lymphadenectomy, Radiation therapy may also be given after surgery, Radiation therapy before and after surgery, or A clinical trial of surgery followed by chemotherapy. Treatment of stage IV adult soft tissue sarcoma that involves internal organs of the body may include the following: Surgery (wide local excision), Surgery to remove as much of the tumor as possible followed by radiation therapy, or High-dose radiation therapy, with or without chemotherapy, for tumors that cannot be removed by surgery; Chemotherapy with 1 or more anticancer drugs, before surgery or as palliative therapy to relieve symptoms and improve the quality of life, a clinical trial of chemotherapy with or without stem cell transplant, or a clinical trial of chemotherapy following surgery to remove cancer that has spread to the lungs. These treatments may be followed by surgery to remove lesions on the lungs.