Understanding Sarcoma: Types, Treatment and Living Well

Sarcoma is a rare and complex type of cancer that forms in connective tissues, which include bones, muscles, fat, tendons, blood vessels and nerves. Unlike most cancers that begin in organs such as the lungs or breast, sarcomas start in the body’s structural tissues. Because they can develop almost anywhere, diagnosis and treatment often require a specialized, multidisciplinary team.

What are the Types of Sarcomas?

There are two broad categories of sarcoma: soft tissue sarcoma and bone sarcoma.

Soft tissue sarcoma can occur in nearly any part of the body, most commonly in the arms, legs, chest or abdomen. There are more than 50 subtypes, including:

• Liposarcoma: arises from fat cells, often in the thigh or retroperitoneum (the area behind the abdominal organs).
• Leiomyosarcoma: begins in smooth muscle tissue, which lines internal organs and blood vessels.
• Angiosarcoma: develops in the lining of blood or lymph vessels and can spread quickly.
• Synovial sarcoma: often occurs near joints in the arms or legs and is most common in young adults.

Bone sarcoma begins in the bone itself. The two most recognized subtypes are:

• Osteosarcoma: typically affects teenagers and young adults and is most often found in the long bones, such as the thigh or shin.
• Ewing sarcoma: often develops in the pelvis, ribs, or long bones and is more common in children and adolescents.
• Chondrosarcoma: forms in cartilage and tends to affect adults.

How Does Sarcoma Staging Look Like?

Staging describes how advanced the sarcoma is and helps guide treatment planning. It takes into account the tumor’s size, grade and whether it has spread.

• Stage 1: The tumor is low-grade and localized, meaning it hasn’t spread to nearby lymph nodes or distant organs.
• Stage 2: The tumor is larger or higher-grade, suggesting a greater chance of spreading.
• Stage 3: Cancer cells may have reached nearby lymph nodes or tissues.
• Stage 4: The sarcoma has metastasized, or spread, to distant organs such as the lungs or liver.

Because sarcoma is uncommon, accurate staging often requires evaluation by a sarcoma specialist who can interpret imaging and biopsy results.

Genetics Involved in Sarcomas

Genetic changes play a key role in many sarcomas. Some are caused by specific chromosomal translocations, where pieces of DNA swap places and form new fusion genes that drive tumor growth. Others are linked to inherited genetic syndromes, such as:

• Li-Fraumeni syndrome, caused by mutations in the TP53 gene.
• Neurofibromatosis type 1 (NF1), which increases the risk of certain soft tissue sarcomas.
• Retinoblastoma (RB1) mutations, which can increase risk for osteosarcoma.

However, most sarcomas occur sporadically, without a clear inherited component. Genetic testing may help determine treatment options and identify whether family members could be at risk.

What to Expect for a Sarcoma Diagnosis

Because sarcoma can appear in many locations and mimic benign growths, diagnosis can be challenging.

Testing

The diagnostic process usually begins with imaging studies:

• X-rays are useful for identifying bone sarcomas.
• MRI provides detailed images of soft tissue and helps determine the tumor’s relationship to nearby structures.
• CT scans can show whether cancer has spread to the lungs or other organs.

A biopsy—the removal of a small tissue sample — is essential for confirming the diagnosis. The sample is reviewed by a pathologist experienced in sarcoma, who determines the subtype and grade. The tumor’s grade reflects how abnormal the cells look and how quickly they are likely to grow and spread.

Patients should ask whether their biopsy and imaging will be reviewed by a sarcoma specialist or at a comprehensive cancer center with expertise in these rare cancers.

How to Treat Sarcoma

Treatment depends on the sarcoma’s type, location, grade, and stage, as well as the patient’s overall health and goals of care. Many patients benefit from a team approach that includes oncologists, surgeons, radiation oncologists and rehabilitation specialists.

Surgery

Surgery remains the mainstay of treatment for localized sarcoma. The goal is to remove the entire tumor with clear margins, meaning no cancer cells are left behind.

For bone or limb sarcomas, surgeons often perform limb-sparing procedures to avoid amputation whenever possible. In some cases, reconstructive surgery or bone grafts may be needed to restore function.

Radiation Therapy

Radiation therapy may be used before surgery to shrink the tumor and make it easier to remove, or after surgery to destroy remaining cancer cells. Radiation can also relieve pain or control symptoms when sarcoma cannot be fully removed.

Newer radiation techniques, such as intensity-modulated radiation therapy (IMRT) or proton therapy, allow for more precise targeting while minimizing damage to nearby healthy tissue.

Chemotherapy

Chemotherapy uses drugs that travel through the bloodstream to destroy cancer cells. It may be used before surgery to shrink tumors, after surgery to reduce recurrence risk or for metastatic disease.

Certain subtypes, such as Ewing sarcoma or osteosarcoma, are more sensitive to chemotherapy. Common drugs include doxorubicin, ifosfamide and dacarbazine. Oncologists tailor regimens to each patient’s diagnosis and tolerance.

Targeted Therapy and Immunotherapy

Some sarcomas respond to targeted therapies, which block specific genetic changes that drive cancer growth. For example, gastrointestinal stromal tumors (GISTs) often respond to targeted therapy that inhibits the KIT or PDGFRA proteins.

Immunotherapy, which activates the body’s immune system to attack cancer cells, has shown promise for certain sarcoma types and is an active area of research. Clinical trials may offer access to newer options not yet available through standard treatment.

Side Effect Management

Treatment for sarcoma can cause side effects such as fatigue, nausea, pain and changes in mobility. Physical therapy, pain management and nutritional support can improve recovery and quality of life.

Patients should work closely with their care team to report symptoms early. Managing side effects proactively can prevent complications and support long-term well-being.

Emotional Well-Being

A sarcoma diagnosis can be emotionally taxing for patients and loved ones. Support from mental health professionals, patient navigators or cancer support groups can help manage anxiety and uncertainty throughout treatment.

Survivorship

After treatment, survivorship care focuses on monitoring for recurrence, managing long-term side effects and promoting healthy living. Follow-up care usually includes regular physical exams and imaging, especially during the first few years when recurrence risk is highest.

Patients are encouraged to discuss fertility, employment, exercise and nutrition with their oncologist or survivorship team.

Recurrence

Sarcoma can return at the original site or spread to distant organs, most often the lungs. The likelihood of recurrence depends on tumor grade, size and surgical margins.

If sarcoma returns, additional surgery, radiation or systemic therapy may be recommended. Ongoing surveillance and prompt reporting of new symptoms — such as persistent cough, pain or swelling — can allow for earlier detection and intervention.

Editor’s Note: This guide is designed to be a starting point. Your personal experience will be unique. By using this information as a foundation for your discussions, you can partner with your oncologist to make the best decisions for your health.

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