Sarcoma

Sarcoma is a type of cancer that develops in the bones and soft tissues of the body. Soft tissues are those that support and connect other structures, like muscles, fat, tendons, and blood vessels. While uncommon, making up only 1% of adult cancers, it’s important to be aware of the signs and symptoms.

Where Can Sarcoma Occur?

Sarcoma can develop almost anywhere in the body, from the head and neck to the toes. The most common locations include:

• Legs, ankles, and feet (40%)
• Shoulders, arms, wrists, and hands (15%)
• Trunk, chest wall, abdomen, and pelvis (30%)
• Head and neck (15%)

Different Types of Sarcoma

There are over 70 known subtypes of sarcoma, broadly categorized into two main groups:

• Bone Sarcomas: These cancers start in the bones themselves. More than a third of them are diagnosed in people younger than 35, with many occurring in children. Common types include osteosarcoma, chondrosarcoma, and Ewing’s sarcoma.
• Soft Tissue Sarcomas: These cancers begin in the connective tissues like muscles or fat. Unlike bone sarcomas, they are more frequent in adults, although some types like rhabdomyosarcoma occur mostly in children. Examples include liposarcoma (in fat tissue) and leiomyosarcoma (in smooth muscle).

Who gets sarcoma?

Sarcoma can affect both children and adults. Here’s a breakdown of who might be more susceptible:

• Age: Soft tissue sarcoma is more common in adults, while bone sarcoma is more frequent in children, teens, and adults over 65.
• Sex: Bone sarcoma is slightly more common in people assigned male at birth.
• Race: Bone sarcoma is more frequent in Black and Hispanic individuals.

Causes of Sarcoma

Sarcoma, a type of cancer, can develop in your bones and soft tissues like muscles, fat, nerves, and blood vessels. It occurs when changes (mutations) happen in the DNA of immature cells, causing them to grow uncontrollably and form tumors. These tumors can invade nearby healthy tissues and, if left untreated, spread (metastasize) to other parts of the body.

Risk factor

While the exact cause of these DNA mutations remains unknown, just like with other cancers, certain factors can increase your risk of developing sarcoma. Here’s a breakdown:

• Exposure to Chemicals: Certain chemicals used in plastics (vinyl chloride monomer), weed killers (phenoxyacetic acid), and wood preservatives (chlorophenols) have been linked to sarcoma risk.
• Radiation Exposure: High doses of radiation from previous cancer treatment can increase the risk.
• Lymphedema: Chronic swelling in your arms or legs is a potential risk factor.
• Genetic Conditions: Some inherited disorders like Gardner syndrome, Werner syndrome, and neurofibromatosis type 1 can raise your risk.

Symptoms of Sarcoma

The symptoms of sarcoma can vary depending on the location of the tumor. In some cases, there might not be any noticeable symptoms in the early stages. Here are some general signs to watch out for:

• Lumps: A new lump that may or may not be painful.
• Pain: Pain in the affected area, particularly in the arms, legs, abdomen, or pelvis.
• Movement Limitations: Difficulty moving an arm or leg due to pain or swelling.
• Weight Loss: Unexplained weight loss can be a symptom.
• Back Pain: Back pain can sometimes be a sign of sarcoma.

It’s important to note that these symptoms can be caused by other conditions as well. If you experience any of these symptoms, it’s crucial to see your doctor to determine the cause.

Diagnosis of sarcoma

Diagnosing sarcoma involves a series of steps to identify the type and extent of the cancer. Here’s an overview of the diagnostic process:

1. History and Physical Examination: Your healthcare provider will begin by gathering your medical history and conducting a physical examination to assess for any signs or symptoms of sarcoma.
2. Special Tests and Imaging:
   • X-ray: This traditional technique uses minimal radiation to capture images of bones and soft tissues, revealing abnormalities.
   • CT Scan: Taking multiple X-ray images at different angles, a CT scan creates detailed cross-sectional views of your body, aiding in pinpointing the tumor’s location and size.
   • MRI Scan: Using powerful magnets and radio waves, an MRI scan generates high-resolution images, offering a more comprehensive view of soft tissues and bones, especially helpful if an X-ray raises concerns.
   • Bone Scan: Injected radioactive material highlights areas of abnormal bone activity, useful in detecting bone sarcomas.
   • Positron Emission Tomography (PET) Scan: This scan utilizes a special sugar tracer that accumulates in highly active cells, like cancer cells. A PET scan can reveal areas of increased activity, potentially indicating a tumor.
3. Biopsy: A biopsy, where a small tissue sample is extracted from the suspected tumor, is crucial for confirming sarcoma and determining its specific type. A pathologist, a specialist in examining tissues, analyzes the sample under a microscope to identify cancer cells and their characteristics. This analysis plays a vital role in tailoring treatment based on the specific type of sarcoma

Staging Sarcoma

Once sarcoma is diagnosed, staging helps assess the extent of the cancer and guide treatment decisions. The TNM classification system is commonly used for staging, which includes:

• T (Tumor): Describes the size and location of the primary tumor.
• N (Node): Indicates whether cancer has spread to nearby lymph nodes.
• M (Metastasis): Indicates whether cancer has spread to other organs.

Additionally, the cancer’s grade (G) provides information about the appearance of cancer cells, with more normal-looking cells generally associated with a better prognosis.

Based on staging criteria and other factors, sarcomas are assigned a stage number (ranging from 1 to 4), with higher numbers indicating more extensive growth or spread of cancer.

It’s important to discuss your cancer stage with your healthcare provider to understand its implications for treatment and prognosis.

Treatments for sarcoma

Several factors influence your treatment plan, such as the type of sarcoma, its size and location, and your overall health. Here’s an overview of the treatment options:

Surgery:

• Wide Local Excision: This surgery aims to remove the entire tumor with a margin of healthy tissue around it, ensuring all cancer cells are eliminated.
• Limb Salvage: Whenever possible, surgeons prioritize saving the affected limb (arm or leg). This might involve removing the tumor and reconstructing the area using metal replacements for joints or bone grafts from other parts of your body.
• Amputation: In some cases, amputation may be necessary to ensure the complete removal of the cancer. This decision is never taken lightly, and your doctor will discuss all options thoroughly.

Radiation Therapy:

• External Beam Radiation: High-energy X-rays are delivered by a machine outside your body to target the cancer cells while minimizing damage to healthy tissues. Advanced techniques ensure precise radiation delivery.
• Internal Radiation (Brachytherapy): For localized sarcomas, radioactive implants placed inside the body (during surgery or through catheters) deliver concentrated radiation directly to the tumor.

Chemotherapy:

• Chemotherapy drugs attack rapidly dividing cancer cells. These medications can be administered intravenously (through a vein) or orally (as pills). Depending on the treatment plan, chemo might be used before surgery to shrink the tumor, after surgery to destroy remaining cancer cells, or in combination with radiation therapy..

Targeted Therapy:

• These drugs target specific vulnerabilities within cancer cells, leaving healthy cells unharmed. Targeted therapy is only effective for certain types of sarcomas with the specific weaknesses the drug targets. Your doctor will determine if this is a suitable option for you.

Immunotherapy (Biologic Therapy):

• Immunotherapy helps your immune system recognize and fight cancer cells more effectively. Cancer cells can sometimes evade the immune system, but immunotherapy can counteract these defenses and allow your body’s natural defenses to target the cancer.

Thermal Ablation:

• In some cases, an interventional radiologist uses a procedure guided by imaging technology (like a CT scan) to destroy the sarcoma by applying extreme heat or cold directly to the tumor mass.

Palliative Care:

• A specialized team of doctors, nurses, social workers, and nutritionists provides palliative care to manage symptoms, and side effects of treatment, and improve your overall quality of life throughout the cancer journey.

Clinical Trials:

• A specialized team of doctors, nurses, social workers, and nutritionists provides palliative care to manage symptoms and side effects of treatment and improve your overall quality of life throughout the cancer journey.

You won’t be alone in this battle. Your care team may include a variety of specialists, such as surgeons, radiologists, geneticists, medical oncologists (cancer specialists), radiation oncologists, pathologists, pediatric specialists (for cancer in children), psychologists, and social workers. They will work together to create the most effective treatment plan. Additionally, geneticists, pathologists, and social workers may be involved to ensure a comprehensive approach to your care.

When to See Your Doctor

Don’t hesitate to schedule an appointment with your doctor if you notice any of these warning signs:

• A lump or mass, even if it’s painless.
• A mass that’s larger than a golf ball (around 5 centimeters).
• Unexplained bone pain, especially if it doesn’t improve on its own and seems unrelated to any injury.

Survival Rates

The statistics below quote five-year survival rates. This is the percentage of people who live at least five years after their sarcoma diagnosis. Here’s a breakdown of the general ranges:

• Soft tissue sarcoma:
  • 15% for cancer that has spread (metastasized)
  • 81% for cancer that hasn’t spread
• Osteosarcoma (bone cancer)
  • 26% for metastasized cancer
  • 77% for non-metastasized cancer

These numbers paint a broad picture, but they don’t consider all the important details that influence your individual outlook. Several factors can significantly impact your prognosis, including:

• Specific sarcoma type: Different sarcoma types have varying survival rates.
• Location of the sarcoma: Where the cancer is located can affect treatment options and outcomes.
• Overall health: Your general health plays a role in how well you tolerate treatment.
• Treatment response: How your body responds to treatment can influence your survival rate.

Who Can Give You the Best Prognosis?

Given the complexities involved, your doctor is the best resource for understanding your personal outlook. They can analyze your specific case, considering the type, location, and other relevant factors, to give you a more accurate prognosis.

Sources

• Memorial Sloan Kettering Cancer Center: “About Soft Tissue Sarcoma.”
• American Cancer Society: “Osteosarcoma.”
• American Society of Clinical Oncology
• Mayo Clinic
• Clevland Clinic
• University of Michigan Comprehensive Cancer Center: “Sarcoma Overview.”
• Emory University: “What is Sarcoma Cancer?”
• National Cancer Institute: “Cancer Classification;” “General Information About Adult Soft Tissue Sarcoma;” and “Adult Soft Tissue Sarcoma Treatment (PDQ), Health Professional Version.”
• “Sarcoma – Adult Soft Tissue Cancer,” American Cancer Society, 2012.
• Penn Medicine: “What causes sarcoma?”