Sarcoma is a rare type of cancer that develops in the bones and soft tissues, including fat, muscles, nerves, fibrous tissues, blood vessels, and deep skin tissues.

Sarcoma can happen anywhere on the body. It can cause lumps that may be painless, but may become painful as they grow. Surgery may be required to treat sarcoma.

Learn more about sarcoma, including its definition, types, causes, symptoms, diagnosis, treatment, and potential risks and complications.

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Surgical oncologists at WashU Medicine see patients with cancer at Siteman Cancer Center, which has received the highest possible rating from the National Cancer Institute.

What is sarcoma?

Sarcoma is a type of cancer that arises from connective tissues in the body. It can occur in any part of the body but is most commonly found in the limbs, chest, and abdomen. Sarcomas are broadly categorized into two main types: soft tissue sarcomas and bone sarcomas.

Types of sarcoma

Soft tissue sarcoma

  • Liposarcoma: Develops in fat tissue.
  • Leiomyosarcoma: Arises from smooth muscle tissue.
  • Rhabdomyosarcoma: Originates from skeletal muscle tissue.
  • Angiosarcoma: Develops in blood vessels or lymph vessels.
  • Synovial sarcoma: Occurs near joints and tendons.
  • Fibrosarcoma: Arises from fibrous connective tissue.
  • Gastrointestinal stromal tumors (GIST): Develop in the walls of the digestive tract.

Bone sarcoma

  • Osteosarcoma: The most common type of bone cancer, typically affecting children and adolescents.
  • Ewing’s sarcoma: Affects bones or the soft tissue around bones and is more common in children and young adults.
  • Chondrosarcoma: Develops in cartilage cells and is more common in adults.

Causes of sarcoma

Genetic factors

  • Inherited syndromes: Certain genetic syndromes, such as Li-Fraumeni syndrome, familial adenomatous polyposis (FAP), and retinoblastoma, increase the risk of sarcoma.
  • Genetic mutations: Mutations in specific genes can lead to uncontrolled cell growth and the development of sarcoma.

Environmental factors

  • Radiation exposure: Previous radiation therapy for other cancers can increase the risk of developing sarcoma.
  • Chemical exposure: Exposure to certain chemicals, such as herbicides, dioxins, and polyvinyl chloride, may be linked to an increased risk of sarcoma.

Other risk factors

  • Chronic lymphedema: Persistent swelling of tissues due to lymphatic system blockages can increase the risk of angiosarcoma.
  • Previous cancer treatments: History of radiation or chemotherapy treatments may elevate the risk of developing sarcoma.

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Symptoms of sarcoma

Soft tissue sarcoma

  • Lump or mass: A painless lump or swelling under the skin, often in the limbs, chest, or abdomen.
  • Pain: Discomfort or pain as the tumor grows and presses on nerves or muscles.
  • Obstruction: Symptoms related to the obstruction of nearby organs, such as bowel obstruction with gastrointestinal stromal tumors.

Bone sarcoma

  • Bone pain: Persistent, worsening pain in the affected bone.
  • Swelling: Swelling and tenderness near the affected area.
  • Fractures: Weakened bones leading to fractures with minimal trauma.

Diagnosis of sarcoma

Medical history and physical examination

  • Symptom assessment: Detailed inquiry about the presence, duration, and severity of symptoms such as lumps, pain, and swelling.
  • Physical examination: Palpation of the affected area to evaluate the size and characteristics of any masses.

Imaging studies

  • X-rays: Initial imaging to detect abnormalities in bones.
  • MRI: Magnetic resonance imaging for detailed images of soft tissue and bone structures.
  • CT scan: Cross-sectional imaging to visualize the extent of the tumor and detect metastases.
  • PET scan: Positron emission tomography to assess the metabolic activity of the tumor and detect metastases.

Biopsy

  • Needle biopsy: A needle is used to extract a tissue sample from the tumor for examination under a microscope.
  • Surgical biopsy: A small surgical procedure to remove a portion of the tumor for diagnostic analysis.

Laboratory tests

  • Genetic testing: Analysis of specific genetic mutations associated with certain types of sarcoma.
  • Blood tests: General blood work to assess overall health and organ function.

Treatment of sarcoma

Surgery

  • Wide local excision: Removal of the tumor along with a margin of healthy tissue to ensure complete excision.
  • Limb-sparing surgery: Removing the tumor while preserving the affected limb, when feasible.
  • Amputation: In cases where the tumor cannot be removed without sacrificing the limb, amputation may be necessary.
  • Reconstructive surgery: Procedures to restore function and appearance after tumor removal.

Radiation therapy

  • External beam radiation: High-energy beams directed at the tumor to kill cancer cells and shrink the tumor.
  • Brachytherapy: Placement of radioactive material directly inside or near the tumor.

Chemotherapy

  • Systemic chemotherapy: Drugs administered orally or intravenously to kill cancer cells throughout the body.
  • Neoadjuvant chemotherapy: Administered before surgery to shrink the tumor and make surgical removal easier.
  • Adjuvant chemotherapy: Given after surgery to kill any remaining cancer cells and reduce the risk of recurrence.

Targeted therapy

  • Tyrosine kinase inhibitors: Drugs like imatinib or pazopanib that specifically target cancer cell growth pathways, particularly for certain types of sarcomas such as gastrointestinal stromal tumors.

Immunotherapy

  • Immune checkpoint inhibitors: Drugs that help the immune system recognize and attack cancer cells.

Potential risks and complications

Treatment-related complications

  • Infection: Risk of infection at the surgical site.
  • Bleeding: Risk of bleeding during and after surgery.
  • Nerve damage: Potential damage to nerves during tumor removal, leading to loss of function or sensation.
  • Radiation side effects: Skin irritation, fatigue, and damage to nearby tissues.
  • Chemotherapy side effects: Nausea, vomiting, fatigue, hair loss, and increased risk of infection.

Long-term complications

  • Recurrence: Risk of sarcoma returning at the original site or spreading to other parts of the body.
  • Metastasis: Spread of the cancer to other organs, such as the lungs or liver.

Impact on quality of life

  • Physical limitations: Potential loss of function or mobility due to surgery or tumor effects.
  • Emotional and psychological impact: Stress, anxiety, and depression related to cancer diagnosis, treatment, and long-term follow-up.

Sarcoma is a rare type of cancer that requires specialized diagnosis and treatment to improve outcomes and quality of life for affected individuals.

Understanding the types, causes, symptoms, diagnostic methods, and treatment options is essential for effective care. If you are diagnosed with sarcoma or have symptoms suggestive of this condition, consult with a specialized medical team to develop a personalized care plan tailored to your specific needs and circumstances.

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