Sarcomas are a rare group of cancers that can occur most anywhere in the body. They can occur in bones or soft tissues like muscle or fat. There are over 80 types of sarcomas; soft tissue sarcomas make up less than 1% of all cancer diagnoses. Due to the rarity of soft tissue sarcomas, it’s important to have a team of experts who understand how to treat them. Not only is the OSUCCC – James among the top sarcoma programs in the country, it’s also the only central Ohio provider for patients with recurrent sarcoma. The James is the main referral center for regional and national clinical trials in soft tissue sarcomas.
What is soft tissue sarcoma?
Soft tissue sarcomas are cancerous tumors that occur in the soft tissues, such as muscles, fat, nerves, blood vessels, connective tissues and very rarely in lymph vessels. These sarcomas can spread via the lymph vessels, which are thin tubes that connect the lymph nodes. In adults, most soft tissue sarcomas form in the arms, legs, head, neck, trunk, abdomen and pelvis.
Types of soft tissue sarcomas
Soft tissue sarcomas are classified according to the types of cells in which they begin. There are several types of soft tissue sarcomas.
They include:
- Adipocytic (fat containing) tumors
- Fibroblastic/myofibroblastic tumors
- So-called fibrohistiocytic tumors
- Smooth muscle tumors
- Skeletal muscle tumors
- Vascular tumors
- Tumors of peripheral nerves
- Chondro-osseous tumors
- Tumors of uncertain differentiation
Soft tissue sarcoma symptoms
Soft tissue sarcomas may not always have symptoms. Symptoms will depend on the location and size of the suspected tumor.
A soft tissue sarcoma may appear as a painless lump under the skin that doesn’t go away and gets bigger with time, often on an arm or a leg (extremities). Sarcomas that begin in the abdomen or pelvis may not cause signs or symptoms until they grow to a noticeable size.
As the tumor grows and presses on nearby organs, nerves, muscles or blood vessels, signs and symptoms may include:
- Pain
- Swelling
- Difficulty breathing
- Growth of a tumor (mass)
It’s important to remember that just because you have these symptoms doesn’t mean you have a soft tissue sarcoma. There are other causes for these symptoms. Talk to your doctor if you notice any unusual symptoms that don’t go away.
Soft tissue sarcoma risk factors
Anything that increases your chances of soft tissue sarcoma is called a risk factor. Risk factors for soft tissue sarcoma include the following inherited disorders:
- Retinoblastoma
- Neurofibromatosis type 1 (NF1; von Recklinghausen disease)
- Tuberous sclerosis (Bourneville disease)
- Familial adenomatous polyposis (FAP; Gardner syndrome)
- Li-Fraumeni syndrome
- Werner syndrome (adult progeria)
- Nevoid basal cell carcinoma syndrome (Gorlin syndrome)
Other risk factors for soft tissue sarcoma include:
- Past treatment with radiation therapy for certain cancers
- Being exposed to certain chemicals, such as Thorotrast (thorium dioxide), Agent Orange, vinyl chloride or arsenic
- Having swelling (lymphedema) in the arms or legs for a long time
- Paget’s disease
Just because you may have a risk factor doesn’t mean you’ll get soft tissue sarcoma. However, having certain risk factors may increase your risk of developing the disease, so it’s important to talk to your doctor to understand your risk.
Diagnosing soft tissue sarcomas
If it’s suspected that you may have a soft tissue sarcoma, experts at the OSUCCC – James will use a multidisciplinary approach to diagnose and treat, if applicable, your specific sarcoma type.
Doctors may order a series of imaging tests to help them understand the tumor size and location as well as order biopsies for further evaluation.
Imaging tests used for diagnosis include:
- Computed tomography (CT) scan
- Magnetic resonance imaging (MRI) scan
- Positron emission tomography (PET) scan
- X-ray
- Ultrasound
In addition to ordering imaging tests, you may undergo a biopsy as well. A doctor may use a needle to take a sample of tissue from the suspected tumor. An OSUCCC – James pathologist will then examine that tissue sample under a microscope. They will classify the tumor and determine whether the tumor is or isn’t cancerous based on their findings. Should it be a soft tissue sarcoma, treatment options will be shared with you following the results.
In a biopsy, a doctor removes a small sample of tissue from the mass to be analyzed under a microscope for signs of cancer. There are several ways to obtain a biopsy sample for diagnosing soft tissue sarcoma:
- Incisional biopsy: The doctor removes a tissue sample during a surgical procedure.
- Needle biopsy: The doctor uses a needle to remove a small sample of tissue.
- There are two types of needle biopsy: fine and core. Core uses a wider needle than a fine needle biopsy.
- Excisional biopsy: An experienced OSUCCC – James surgeon removes an entire lump or area of abnormal tissue. The sample will undergo further testing following the biopsy procedure to help the OSUCCC – James experts classify the type and subtype of soft tissue sarcoma. This type of biopsy should only be performed by a surgeon with expertise in sarcoma as complications can lead to worse outcomes and/or more difficult treatment options.
These tests include:
- Immunohistochemistry: This test uses dye on proteins called antibodies to identify certain antigens in the tissue sample. Antigens are any substance that causes a response in the body’s immune system against that substance. Antigens include toxins, bacteria, chemicals, viruses or other substances that come from outside the body.
- Light and electron microscopy: Changes in cells that indicate cancer types are evaluated under high-powered microscopes.
- Cytogenetic analysis: This test analyzes the cells to identify changes in the chromosomes.
- Fluorescence in situ hybridization (FISH): This laboratory test analyzes the cells’ genes or chromosomes. A special light and fluorescent dye cause certain genes or specific areas of chromosomes to light up when pieces of lab-created DNA attach to them.
- Flow cytometry: Flow cytometry measures numbers and proportions of cells in a soft tissue sample and can determine characteristics of cells, such as their shape, size and presence of tumor markers. Tumor markers are substances that indicate certain cancers.
Soft tissue sarcoma treatment options
After a soft tissue sarcoma diagnosis is confirmed, you may be wondering about what treatment options you might have. At the OSUCCC – James, we have full-time staff dedicated to treating soft-tissue sarcomas. Our team of specialists and subspecialists, including surgical oncologists, radiation oncologists, medical oncologists and others, use a multidisciplinary approach to treating sarcomas.
We know that your cancer is different and no two sarcomas are the same. First, your doctor will perform a physical exam. They will ask questions about your health history as well as examine your body for additional lumps or signs of sarcoma and determine your overall health.
From there, one or more of the following treatment therapies may be recommended for your specific case:
Surgery
Most patients who have soft tissue sarcoma have surgery. Surgery may be the only treatment necessary for some soft tissue sarcomas.
Surgery may consist of one of the following procedures:
- Local excision and/or limb-sparing surgery: An OSUCCC – James surgical oncologist removes the soft tissue tumor and some normal tissue around the tumor. The surgery also is called wide local excision. You may receive radiation therapy or chemotherapy before surgery to shrink the tumor. A graft of your healthy tissue or bone (or artificial materials) may be needed to replace tissue and bone removed during surgery.
- Amputation: In rare cases, a surgeon must remove a portion of or all of your arm or leg to treat soft tissue sarcoma.
Radiation therapy
Radiation therapy uses high-energy X-rays or other radiation to kill cancer cells or keep them from growing.
If you have soft tissue sarcoma, you may receive radiation therapy before or after surgery to remove a tumor. Radiation can shrink a tumor to make surgery more successful. Postoperative radiation therapy is used to destroy any cancer cells that may remain.
The OSUCCC – James radiation experts are leaders in innovative radiation treatments, and The James is one of the few hospitals in Ohio to offer radiation treatments in the prone position (laying down).
The OSUCCC – James also offers other leading-edge radiation treatments, including proton therapy, imaging-based radiation therapy, conformal therapy, intensity-modulated radiation therapy (IMRT) and stereotactic radiosurgery, and using a Varian TrueBeam linear accelerator.
Conformal therapy, a type of radiation therapy, uses a computer to create a three-dimensional picture of the tumor. This allows the OSUCCC – James specialists to hit the tumor with the highest possible dose of radiation while sparing surrounding normal tissue as much as possible. This therapy is also sometimes called three-dimensional radiation therapy or 3D-CRT.
Chemotherapy
Chemotherapy drugs stop cancer cell growth by either destroying the cells or stopping them from dividing.
When chemotherapy is given before surgery, it can shrink a soft tissue sarcoma tumor. After surgery, it can lower the risk that the soft tissue sarcoma will return or spread to other sites in the body. Chemotherapy drugs can be taken by mouth or injected into a vein, and they may be given over a period of months.
When the drugs enter the bloodstream, they can reach cancer cells throughout the body (called systemic chemotherapy). Combination chemotherapy uses more than one anti-cancer drug.
Chemotherapy treatment can take place in an outpatient part of the hospital. You may need to stay in the hospital for IV treatment. Oral medication chemotherapy is given for trial therapy, which can be used for either recurrent diseases or for sarcoma clinical trials.
Clinical trials
Depending on your soft tissue sarcoma type, you may be eligible to participate in a clinical trial. The OSUCCC – James currently has over 20 clinical trials underway for different types of sarcoma treatments. You can participate in clinical trials before, during or after starting your cancer treatment. To determine if you are eligible, your doctors will discuss options with you.
If you have been diagnosed with soft tissue sarcoma, would like a second opinion or would like to speak with a sarcoma specialist, please call The James Line at 800-293-5066 or 614-293-5066 to make an appointment.
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