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What is Sarcoma Cancer?

What is Sarcoma?

Sarcoma is a rare tumor that accounts for approximately 1% of all adult cancers. Sarcoma comes from the Greek word “sarx,” meaning “flesh,” and it arises from the embryonic mesoderm; specifically, the tissue in bone, around joints, fat, muscle, nerve, or blood vessels and can develop in any part of the body—head and neck, truncal, upper and lower extremities, gastrointestinal tract, and retroperitoneum.

There are over 50 histological types of sarcomas and appropriate classification is essential as prognosis differs significantly and individual tumors may benefit from different treatment strategies. For discussion and treatment purposes, sarcomas can be grouped into two main categories of sarcoma, those that arise from bone and those from soft tissues.

Bone:  The most common type of sarcoma from the bone is osteosarcoma. Other types include Ewing's sarcoma and chondrosarcoma (arising from cartilage). Bone sarcomas are rare tumors that occur most commonly in children and young adults but can also occur at any age.

Soft tissue:   Soft tissue sarcomas (STS) encompass tumors that arise from the fat, muscle, nerves, and blood vessels that are present throughout our entire body. STS most often occur in the extremities (arms or legs), but can occur in other areas such as the torso, abdomen, retroperitoneum, and gastrointestinal tract (i.e. GIST).

Common types of STS are:

  • Fat
    • Liposarcoma
    • Atypical lipomatous tumor
  • Muscle
    • Leiomyosarcoma
    • Rhabdomyosarcoma
    • Pleomorphic undifferentiated sarcoma
  • Nerve
    • Schwannoma
    • Neurofibroma
    • Malignant peripheral nerve sheath tumor
  • Blood vessel
    • Hemangioendothelioma
    • Angiosarcoma
    • Kaposi sarcoma

How Does Sarcoma Present?
Sarcomas of the extremities and torso may present as a lump that is not always painful. Sarcomas located in the abdomen or retroperitoneum can cause pain or problems caused by the blockage of the intestines, but symptoms are often not specific to sarcoma.