Sarcoma: Types, Risk Factors, and Advances in Treatment
- Yhaimar Barile
- Jul 16
- 5 min read
In this article:
What is sarcoma?
How rare Is sarcoma?
What causes sarcoma?
What are the risk factors for sarcoma?
What are the types of sarcoma?
What are the symptoms of sarcoma?
When should you see a doctor?
How Is sarcoma diagnosed?
What are the stages of sarcoma?
How Is sarcoma treated?
What is the outlook for sarcoma?
Overview
What Is Sarcoma?
Sarcoma is a rare type of cancer. It is a malignant tumor that starts in the connective tissues of the body. Connective tissue supports and connects structures in the body. These tissues include bone, muscle, fat, nerves, blood vessels and tissue around joints. Sarcoma does not begin in organs or glands, different from most cancers. Mayo Clinic
How Rare Is Sarcoma?
Sarcoma is uncommon. In the United States, soft tissue sarcomas make up 1% of cancers in adults and 15 % of cancers in children. Each year, physicians diagnose about 13,000 soft tissue sarcomas and 4,000 bone sarcomas. For context, breast cancer alone accounts for more than 300,000 new cases annually.
What Causes Sarcoma?
The exact cause for most sarcomas is still unknown. These cancers begin DNA changes occur in connective tissue cells. But for most people, the reason for these changes is not clear. Only a minority of cases link directly to genetic syndromes, radiation, or chemical exposure. Mayo Clinic American Cancer Society
What Are the Risk Factors for Sarcoma?
Several factors increase the likelihood from developing sarcoma:
Genetic syndromes: Some inherited conditions elevate sarcoma risk, including familial retinoblastoma, Li-Fraumeni syndrome, and neurofibromatosis.
Radiation: Previous radiation therapy for other cancers increases sarcoma risk.
Lymphedema: Long-term swelling of the lymphatic system raises the chances of developing sarcoma.
Chemicals: Contact with certain chemicals, like those used to make plastic (vinyl chloride), herbicides (phenoxyacetic acid), wood preservatives (chlorphenols), or arsenic may raise the risk.
Viruses: Some viruses, like the one that causes Kaposi sarcoma, elevate risk, especially in people with weak immune systems.
Learn more at the Mayo Clinic and Cleveland Clinic.
What Are the Types of Sarcoma?
Sarcoma is divided into categories based on the specific connective tissue in the body where it starts. Medical professionals recognize more than 70 subtypes of sarcoma, each with distinct characteristics and treatments.
Soft Tissue Sarcoma
Soft tissue sarcomas begin in tissues like muscle, fat, nerves, blood vessels, and deep skin layers. Sarcoma can form almost anywhere in the body, most commonly in the arms, legs, chest, or abdomen. Both adults and children develop soft tissue sarcoma.
Bone Sarcoma
Bone sarcomas start in the bone tissue. These malignancy occur mostly in children and young adults but can develop at any age. Common types include osteosarcoma and Ewing sarcoma.
Symptoms
What Are the Symptoms of Sarcoma?
Symptoms vary depending on where the tumor’s location. Early-stage sarcoma may not cause symptoms, while others cause pain only when the tumor grows large enough and pressures a nerve, muscle, or organ.
Soft tissue sarcoma symptoms may cause:
• A lump that may or may not be painful
• Progressive swelling that increases over time.
• Abdomen or weight loss (for sarcomas in the abdomen)
Bone sarcoma symptoms may include:
• Bone pain and/or swelling
• Bone fractures without a clear cause
• Limited joint mobility
• Unexplained fever
Sarcoma symptoms are often like other conditions. Your doctor can determine if your symptoms relate to sarcoma or another disease.
When Should You See a Doctor?
Consult a doctor if you notice a lump that grows, causes pain, or is larger than a golf ball. Also, get medical attention for persistent bone pain, swelling or any unusual symptoms that don’t go away. Early diagnosis can improve treatment options and outcomes.
Diagnosis and Tests
If sarcoma symptoms appear, your doctor may order several tests to find out more.
How Is Sarcoma Diagnosed?
Physicians use several key steps to diagnose sarcoma:
Physical Examination: Your doctor will ask about your symptoms and medical history. They will check for lumps, swelling, or tenderness.
Imaging Tests: MRI, CT scans, PET scans, or X-rays help locate the tumor and show potential spread.
Biopsy: The doctor removes a small tissue sample, examine under a microscope to confirm if it is sarcoma.
Your doctor may order one or more of these tests based on your symptoms and what they find during your exam. Completing all the needed tests may take several days.
What Are the Stages of Sarcoma?
Doctors use staging to understand how advanced the sarcoma is and guide plan treatment. Staging shows whether the cancer has spread and how far.
Localized: Cancer is only in the original area.
Regional: Cancer has spread to nearby tissues or lymph nodes.
Metastatic: Cancer has spread to distant parts of the body.
Doctors may also use the TNM system:
T (Tumor): Describes the size and location of the main tumor.
N (Nodes): Shows whether the cancer has spread to nearby lymph nodes.
M (Metastasis): Indicates whether the cancer has spread to other organs.
Sarcoma stages typically range from 1 to 4. A higher number means the cancer is more advanced disease or further spread. There are other staging methods depending on the sarcoma type. Ask your doctor about your specific stage, treatment options, and what to expect.
Management and Treatment
How Is Sarcoma Treated?
Treatment depends on the tumor’s type, size, location and stage. Main options include:
Surgery: The primary treatment for most sarcoma. The goal is to remove the tumor completely. In some cases, surgeon must remove a limb (amputation) to remove all cancer cells. Surgeons try to save the lamb when possible.
Radiation Therapy: This treatment targets sarcoma with specialized energy beams from protons, x-rays or other sources. Doctors often used radiation before or after surgery to shrink the tumor or kill remaining cancer cells.
Chemotherapy: This treatment uses powerful medicine to fight cancer. Certain sarcomas respond well to chemotherapy, especially when the cancer has spread. Doctors may give patients chemotherapy before or after surgery.
Targeted Therapy: These treatments attack specific chemicals in sarcoma cells. They block cancer cell growth and trigger cell death. Doctors may test your sarcoma to see if targeted therapy will prove effective, especially for recurrent or metastatic disease.
Immunotherapy: Immunotherapy increases the ability of your immune system to find and kill cancer cells. Cancer cells can hide from the immune system. Immunotherapy helps your immune cells recognize and destroy these hidden cancer cells. Doctors can test your sarcoma to see if it will respond to immunotherapy. This treatment may help control sarcoma that has spread to other parts of your body.
Ablation therapy: This procedure directly destroys sarcoma cells using a a variety of energy forms. Some ablation types use radiofrequency or microwave energy to heat and kill the cancer cells. Cryoablation uses extreme cold to freeze and destroy the cells. Doctors may use ablation to treat sarcoma when surgery is not possible or when cancer has spread to other parts of the body.
Outlook and Prognosis
What Is the Outlook for Sarcoma?
The outlook for sarcoma patients depends on the type, size, location, and stage of the tumor, and how well the cancer responds to treatment. When found early, many sarcomas respond successfully to treatment. The five-year survival rate for soft tissue sarcoma is about 65%, but survival rates are higher when the cancer is found before it has spread.
References
American Cancer Society
Cleveland Clinic
https://my.clevelandclinic.org/health/diseases/17934-sarcoma
Johns Hopkins Medicine
https://www.hopkinsmedicine.org/health/conditions-and-diseases/sarcoma
Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/sarcoma/symptoms-causes/syc-20351048
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