Sarcoma

A sarcoma (from the Greek 'sark' meaning "flesh") is a cancer of the connective or supportive tissue (bone, cartilage, fat, muscle, blood vessels) and soft tissue. This is in contrast to carcinomas, which are of epithelial origin (breast, colon, pancreas, and others).

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Classification

Sarcomas are given a number of different names, based on the type of tissue from which they arise. For example, osteosarcoma arises from bone, chondrosarcoma arises from cartilage, and leiomyosarcoma arises from smooth muscle. Sarcomas strike people in all age ranges, but they are very rare, accounting for only 1% of all cases of cancer.^[1] Soft tissue sarcomas, such as leiomyosarcoma, chondrosarcoma, and gastrointestinal stromal tumor (GIST), are more common in adults than in children. GIST is the most common form of sarcoma, with approximately 3000-3500 cases per year in the United States.^[2] This should be compared with breast cancer, with approximately 200,000 cases per year in North America.^[3] Bone sarcomas, such as osteosarcoma and Ewing's sarcoma, are more common in children than in adults. These tumors most commonly strike adolescents and young adults between the ages of 12 and 25. In addition to being named based on the tissue of origin, sarcomas are also assigned a grade, such as low grade or high grade. Low grade sarcomas are usually treated surgically, although sometimes radiation therapy or chemotherapy are used. High grade sarcomas are more frequently treated with chemotherapy. Since these tumors are more likely to undergo metastasis (spreading to distant sites), these tumors are treated more aggressively. Childhood sarcomas are almost always treated with a combination of surgery and chemotherapy, and radiation is frequently used as well. The recognition that childhood sarcomas are sensitive to chemotherapy has dramatically improved the survival of patients. For example, in the era before chemotherapy, long term survival for patients with localized osteosarcoma was only approximately 20%, but now has risen to 60-70%.^[4]

Organizations

Private funding Organizations such as the 'Sarcoma Foundation of America' are working to fund research toward a cure for sarcoma, while other patient-oriented organizations such as the Sarcoma Alliance are working to educate patients about the disease, and still others like the Rare Cancer Alliance are working to give patients online support. The Liddy Shriver Sarcoma Initiative publishes the Electronic Sarcoma Update Newsletter, which is an online bi-monthly newsletter, containing articles of interest to patients, caregivers, physicians, nurses, and survivors. Information on clinical trials in sarcoma can be found through the National Cancer Institute. National cooperative groups (ECOG, SWOG, ACOSOG, etc.) conduct clinical trials in sarcomas, as do investigators in the Sarcoma Alliance for Research through Collaboration (SARC).

Notable patients

Actor Robert Urich had a soft-tissue sarcoma, and died of this disease. Actress Michelle Thomas died from a rare abdominal soft-tissue sarcoma. The most famous victims of this disease are Canadian Terry Fox, who was required to have a leg amputated as treatment, and the Soviet mathematician Nikolai Kochin who perished at the age of 43. Fox subsequently began heroic fundraising efforts towards medical research, which enabled advances that dramatically improved the survivability of the ailment.

Types of sarcoma

(ICD-O codes are provided where available.)

• Soft tissue sarcoma, including
  • Alveolar soft part sarcoma (9581/3)
  • Angiosarcoma (9120/3)
  • Cystosarcoma Phylloides[1]
  • Dermatofibrosarcoma (8832/3-8833/3)
  • Desmoid Tumor (8821/1-8822/1)
  • Desmoplastic small round cell tumor (8806/3)
  • Epithelioid Sarcoma (8804/3)
  • Extraskeletal chondrosarcoma (9220/3)
  • Extraskeletal osteosarcoma (9180/3)
  • Fibrosarcoma (8810/3)
  • Hemangiopericytoma (9150)
  • Hemangiosarcoma (9120/3)
  • Kaposi's sarcoma (9140/3)
  • Leiomyosarcoma (8890/3-8896/3)
  • Liposarcoma (8850/3-8858/3)
  • Lymphangiosarcoma (9170-9175)
  • Lymphosarcoma
  • Malignant fibrous histiocytoma (8830/3)
  • Neurofibrosarcoma (9540/3)
  • Rhabdomyosarcoma (8900-8920)
  • Synovial sarcoma (9040/3-9043/3)
• Askin's Tumor (8803/3)
• Ewing's (9260/3) - PNET (9473/3)
• Malignant Hemangioendothelioma (9130/3)
• Malignant Schwannoma (9560/3-9561/3)
• Osteosarcoma (9180/3-9190/3)
• Chondrosarcoma (9220/3-9240/3)

References