What is Sarcoma?

Sarcoma is a rare cancer — accounting for just 1 percent of adult cancers — and it arises from the tissues that glue the body together — bone, muscle, fat, tendons, blood vessels, lymph vessels, nerves and joint tissue.

Sarcomas usually occur in the extremities, abdomen and trunk. About half of sarcomas occur in arms and legs and about 20 percent develop in the abdomen or trunk. Because the body has these tissues everywhere, sarcomas can occur anywhere, and can take many forms. Superficial sarcomas lie within or directly underneath the skin. Others develop deep within the muscle, the chest or abdomen. The tumor may be as small as a pea (1 centimeter) to as large as a beach ball (40 centimeters).

Types of sarcomas

More than 50 subtypes of sarcoma are currently recognized. Each tumor subtype displays a unique behavior, has a unique biology and requires a unique treatment — making it important to seek personalized care from physicians who specialize in sarcoma. The Roswell Park sarcoma team diagnoses and treats:

  • Soft tissue sarcomas (of any location or organ)
  • Gastrointestinal Stromal Tumor (GIST)
  • Desmoid/aggressive fibromatosis
  • Dermatofibrosarcoma protuberans (DFSP)
  • Atypical lipomas or intramuscular lipomas
  • Neurogenic tumors (neurofibroma and schwannoma)
  • Solitary fibrous tumor
  • Ganglioneuroma
  • Adrenal myelolipoma
  • Other unusual benign tumors such as: elastofibroma, hemangioma and lymphangioma

Sarcoma Diagnosis

The Roswell Park take

Most physicians, including surgeons will see only one or two patients with a sarcoma in their entire career. Our sarcoma team handles more than 200 patients a year, dedicating each day to the care of patients with soft tissue sarcoma.

Our treatment approach to sarcoma has three goals:

  • Minimize chance of recurrence or spread
  • Preserve function for highest quality of life
  • Optimal outcome for survivorship

Risk factors of sarcoma

A risk factor is anything that may increase your risk for a disease. Risk factors for soft tissue sarcoma include:

  • Radiation exposure or treatment during childhood
  • Previous radiation treatment for retinoblastoma, lymphoma, or breast, cervical cancer or gastrointestinal cancer
  • Exposure to toxic chemicals such as herbicides, pesticides and Agent Orange

Having certain inherited disorders may increase your risk for sarcoma:

  • Retinoblastoma: a cancer that forms in the tissues of the retina - usually occurring in children younger than 5 years old.
  • Neurofibromatosis type 1(von Recklinghausen disease or NF1): a rare genetic condition that causes brown spots and tumors on the skin.
  • Tuberous sclerosis: a genetic disorder in which noncancerous tumors form in the kidneys, brain, eyes, heart, lungs, and skin.
  • Familial adenomatous polyposis (FAP): an inherited condition in which numerous polyps (growths that protrude from mucous membranes) form on the inside walls of the colon and rectum.
  • Li-Fraumeni syndrome: a rare, inherited predisposition to multiple cancers due to a mutation in the tumor-suppressant gene TP53.
  • Werner syndrome: an inherited disorder marked by rapid aging that begins in early adolescence, caused by a mutation in a gene involved in cell division.
  • Basal cell nevus syndrome: a genetic condition that causes unusual facial features and disorders of the skin, bones, nervous system, eyes, and endocrine glands.

Symptoms of sarcoma

Sarcomas are often first noticed as an odd lump or swelling under the skin, which may or may not be painful. They do not have a precancerous phase or show early warning signs. No screening test yet exists to detect sarcoma early. Many are detected incidentally, during testing or scans performed for other conditions or symptoms. Sarcoma that begins in the abdomen may not cause any symptoms until it grows larger and presses on nearby organs, blood vessels and nerves. The sarcoma may cause pain or trouble breathing