Sarcoma Diagnosis &
Treatment
Sarcoma
Diagnosis & Treatment
A sarcoma diagnosis can feel overwhelming, and it is completely natural to have questions about what comes next. Understanding how sarcoma is diagnosed and the treatment options available can help you and your family feel more prepared as you move forward. Rein in Sarcoma offers educational resources and real patient stories to support you through diagnosis, treatment, and recovery.
1%
Of all new cancer diagnoses
in the U.S. are sarcoma
83%
5-year survival rate for patients with localized sarcoma
17K
Newly diagnosed patients each
year in the United State
The Diagnostic
Process
Early and accurate diagnosis is an important part of effective care. Because sarcomas are rare and can develop in many different parts of the body, reaching the correct diagnosis often involves several tests and the insight of specialists who understand these cancers.
Grading
The “grade” of your tumor refers to how your cancer cells look under a microscope compared to normal healthy cells, as typically described in a pathology report.
Staging
The “stage” of your tumor refers to where cancer is located in the body, ranging from local (in one place) to metastatic (spread).
Testing
Each person’s cancer has unique genetic and molecular markers that can be identified through genomic testing after a biopsy or surgery.
Grading
The “grade” portion of your tumor is about appearance and describes how much of your cancer cells within your tumor look like normal healthy cells when under a microscope. This information is usually found in pathology reports.
Sarcoma tumors have four grades. In general, the lower the grade, the better the prognosis will be.
For example, a grade 1 tumor is in an early stage of growth, may grow very slowly, and be unlikely to spread throughout your body. A grade 3 tumor has cells that are dividing and growing fast and may look very abnormal under the microscope. There are also cells described as undifferentiated or dedifferentiated which are cells growing and dividing very fast and look very, very abnormal.
Staging
The “stage” portion of your tumor is about location and describes all the places the cancer cells or tumors are located in your body. There are several different ways to describe the cancer stage. For some patients, the cancer will be described as “local” (in one place) or “metastatic” (meaning that it has spread in the body).
A universally common staging system that is more detailed is the Tumor, Node, Metastasis (TNM) system, which tells information about:
- Tumor (T): How large is the primary tumor? Where is it located?
- Node (N): Has the tumor spread to lymph nodes? If so, where, and how many?
- Metastasis (M): Has the tumor spread to other parts of your body?
For example, a grade one/stage one cancer tumor is slow growing (grade one) and located completely in one place in your body (stage one).
It can be helpful for you to know and understand your cancer’s grade and stage, as it will guide treatment plan and help you and your team understand risk of spread and recurrence.
Testing
Each person’s cancer has unique genes, proteins, and other markers that cause the cancer to grow.
Looking for these changes is called genomic or genetic testing. It is also commonly called tumor DNA testing, biomarker testing, or genomic testing. This testing can be done after biopsy or surgery to remove your tumor. There are many, many different changes to genes, proteins, and other markers that can be tested for
Genomic testing of tumors is becoming more and more important as scientists learn more about the genetics of cancer and develop new treatments. By knowing what makes your particular cancer grow, your oncologist can determine which treatments may be effective, especially the newer targeted therapies (see below). Genomic testing may also be necessary for you to qualify for clinical trials (see below). The area of genomic testing is rapidly expanding and will hopefully lead to more effective treatments for sarcoma cancers.
A term you may hear about is liquid biopsy. Liquid biopsy is the means by which a person’s blood is examined to find circulating tumor cells, bits of the genetic material of tumor cells, or other markers for their particular cancer. There are a number of challenges with the use of liquid biopsy in rare sarcomas. This is an area of active research.
Common Treatment Options
Sarcoma treatment is personalized for each patient and based on the unique details of the diagnosis, including the type of sarcoma and where it is located. Many people receive a combination of treatments, guided by a team of specialists who collaborate to provide the best care.
Surgery is often the first step in treating sarcoma.
- Some patients will undergo removal of all or most of their sarcoma as a first step in treatment. Others will have a biopsy first, where a portion of the tumor is removed so that the cells can be looked at under a microscope. If a biopsy was performed, further surgery may be done later to remove the rest of the tumor and some healthy tissue around it.
- Removing healthy tissue around your sarcoma tumor may create a “margin” to help ensure that there are no tumor cells left in that area
- Surgery is typically performed by a surgical oncologist or an orthopedic surgeon who specializes in sarcoma.
- Surgery might be the only treatment necessary in some small, low-grade sarcomas, but can also be combined with chemotherapy, radiation therapy, or targeted therapy (see below) in certain tumor types.
Radiation therapy is the use of high-energy X-rays or other ionizing particles to destroy cancer cells.
- Radiation therapy is guided by a radiation oncologist.
- A radiation therapy plan usually consists of a specific number of radiation treatments given over a set period of time.
- Radiation therapy may be done before surgery to help shrink a tumor for easier removal or after surgery to destroy any cancer cells left behind. It can also be used to treat the pain or side effects of tumors that may not be operable or treatable with chemotherapy.
- There are different types of radiation therapy so it’s important to discuss with your radiation oncologist the best type of treatment for you. It’s also important to discuss the specifics of your radiation treatment, including side effects, expectations, etc.
Chemotherapy is medication generally given orally or intravenously that may stop cancer cells from growing and dividing. Chemotherapy may be given prior to surgery to help shrink a tumor for easier removal or after surgery to destroy any cancer cells left behind.
- Chemotherapy may be given alone or in combination with surgery, radiation therapy, or both.
- Chemotherapy treatment “cycles” refers to a specific number of treatments given over a set period of time.
- Chemotherapy is coordinated by an oncologist.
- As there are both short and long-term side effects from having chemotherapy, it is important to discuss this information with your oncologist prior to starting these treatments.
- Many sarcomas are not responsive to chemotherapy.
Targeted therapy drugs treat cancer by acting on parts of cancer cells that help them grow and divide that may be different from normal, non-cancerous cells.
- Targeted therapies may treat cancer by interfering with specific proteins or genetic changes that help tumors grow and spread.
- There are several different types of targeted therapies, and use varies by sarcoma type and stage, so it is best to talk to your oncologist about which treatments may be right for you. They may look for certain changes in genes (mutations) in your cancer that help the cancer cells to grow and divide to determine if targeted therapy will be effective against your specific sarcoma.
- Some targeted therapies are pills that you can swallow, while other medications are given intravenously (through an IV).
- Targeted therapies can work in many different ways. For instance: o They can help the immune system destroy cancer cells.
- They can stop cancer cells from growing and dividing.
- They can stop the cancer cells from forming blood vessels.
- They can deliver cell-killing substances to cancer cells.
- They can prevent cancer cells from getting hormones they need to grow.
- Targeted therapy can have side effects even though they are designed to specifically target cancer cells. Sometimes, healthy non-cancerous cells can be inadvertently targeted as well.
- As research studies continue to identify more targets and develop more targeted therapies, this treatment option is constantly evolving. There may be clinical trials testing new targeted therapy for your sarcoma.
- Example of targeted therapy: o Monoclonal Antibodies: Monoclonal antibodies are proteins designed to attach themselves to specific targets on cancer cells. There are different types of monoclonal antibodies that perform different tasks. One way these can be used in cancer treatment is by attaching a cancer-killing medication to the antibody, so when the antibody binds to the specific target on the cancer cell, it kills the cancer cell.
For additional information, visit The National Cancer Institute at cancer.gov/about-cancer/treatment/types/targeted-therapies
What to Expect During
Diagnosis and Treatment
Every sarcoma journey begins with questions, and understanding what comes next can make the process feel a little less overwhelming. While each person’s experience is different, there are common steps that many patients follow.
During diagnosis, you can expect imaging tests, consultations with specialists, and a biopsy to confirm the type of sarcoma. Your care team will explain each test, why it matters, and what the results may tell them.
It is natural to feel uncertain during this time, but you will not navigate it alone. Many patients find comfort in learning what the process looks like and knowing that specialized support is available at every stage.
Support and Resources
You are not alone. Rein in Sarcoma offers trusted educational materials, patient and family support programs, and opportunities to connect with others who understand the challenges of a sarcoma diagnosis. Whether you are looking for practical information or emotional support, you will find resources designed to help you feel informed, supported, and empowered.
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