Soft tissue sarcomas are a rare type of cancer that arise from the soft tissues of the body.
Soft tissue sarcomas are relatively uncommon, accounting for about 1% of all adult cancers. At AHN, once you are diagnosed with sarcoma, your treatment plan is designed to target your cancer and manage any side effects. Our dedicated care team gives you treatment options including surgery, chemotherapy, immunotherapy, targeted therapy, and radiation therapy.
Soft tissue sarcomas are often painless growing masses than can lack other obvious symptoms. Detecting and diagnosing soft tissue sarcoma can be challenging because the images produced are often not definitive or clear. Sarcoma is a general term for a broad group of cancers that begin in the bones and soft tissues. Soft tissues connect, support, or surround other structures and organs in the body including:
- Muscle
- Fat
- Blood vessels
- Nerves
- Tendons
- Ligaments
- Cartilage
- Synovial tissues (tissues around joints)
AHN Sarcoma Center of Excellence
The AHN Sarcoma Center of Excellence is made up of a team of multiple health care experts walking you through every step of your cancer diagnosis and treatment.
Diagnosis and treatment will usually begin with an appointment with our orthopaedic oncology team. The team works closely with providers amongst different medical specialties including:
- Surgeons
- Medical oncology
- Radiation oncology
- Pathology
- Physical therapy
- Occupational therapy
- Plastic surgery
- Nurse Navigators
At AHN, we strive deliver multidisciplinary care using compassionate and advanced technologies to make your treatment course as painless as possible. We will take care of appointment scheduling, imaging planning, and other aspects of care coordination for our patients so you have time to focus on healing and recovery.
Soft tissue sarcomas symptoms and signs
Since soft tissue sarcomas develop in the cells of the body’s soft tissues, which include muscles, fat, blood vessels, nerves, tendons, and the linings of joints, early symptoms and signs can be hard to spot. The most common areas where soft tissue sarcoma cancer cells develop are in the arms, legs, and belly. Soft tissue sarcoma symptoms and signs can show up as:
- A noticeable lump: This is the most common sign. You might feel a new lump or a lump that's growing. It may or may not be painful. Some can be tender, while others don’t hurt at all.
- Swelling: The area around a lump may swell.
- Pain: As the tumor grows, it can cause pain or be tender to the touch.
Other less common symptoms or signs can show up in the:
- Stomach or abdomen: If the sarcoma is in the belly, you may experience pain, constipation, or feel fuller after a meal than normal.
- Leg or arm: If the sarcoma presses on the nerves or blood vessels, it could cause numbness, tingling, or swelling in the limbs.
It's important to remember that most lumps and bumps are not cancer, but it's good to be aware of the signs and symptoms. If you notice changes or new symptoms, talking with your doctor about them is always a good idea.
Causes and risk factors
In most cases, we don’t know exactly why someone develops a soft tissue sarcoma. It’s not usually caused by something you did or didn't do. Cancer is caused by genetic mutations within cells that make them grow uncontrollably. In some cases, we do know of things that can increase the risk. The risk factors that can play a part in developing sarcoma include:
- Genetic syndromes: Certain inherited genetic conditions, such as:
- Neurofibromatosis type 1 (also called Von Recklinghausen disease)
- Li-Fraumeni syndrome
- Gardner syndrome
- Tuberous sclerosis
- Werner syndrome
- These syndromes are rare, but they increase the risk of various cancers, including soft tissue sarcoma.
- Previous radiation therapy: Having radiation therapy for a previous cancer can slightly increase the risk of developing a soft tissue sarcoma in the treated area later in life.
- Lymphedema: This is swelling caused by a buildup of fluid in the lymphatic system. Chronic lymphedema (long-term swelling) can increase the risk of a specific type of soft tissue sarcoma called angiosarcoma.
- Exposure to certain chemicals: Some chemicals, such as vinyl chloride and dioxin, have been linked to an increased risk of soft tissue sarcoma.
- Age: Soft tissue sarcomas can occur at any age but are more common in older adults.
- Family history: If you have a strong family history of cancer, especially sarcomas, you might have a slightly higher risk.
Soft tissue sarcomas screening and diagnosis
Soft tissue sarcomas are a rare type of cancer that affect the soft tissues of the body, such as muscles, tendons, fat, nerves, and blood vessels. Because of their rarity and diverse presentations, they can be challenging to diagnose. There is no routine screening program for soft tissue sarcomas.
Diagnosing soft tissue sarcomas typically involves a combination of approaches.
Medical history and physical exam
Your doctor will ask about your symptoms, including when they began, their location, any changes, and your family medical history. They will also physically examine you, paying close attention to the area of concern.
Imaging tests
These tests play a crucial role in diagnosing and staging soft tissue sarcomas. They help doctors visualize the tumor, determine its size and location, and assess whether it has spread to other parts of the body.
Common imaging tests include:
- X-ray: May be used to visualize the tumor and its location, but might not be detailed enough for a definitive diagnosis.
- Ultrasound: Can help determine the tumor's size, location, and whether it's solid or filled with fluid.
- Magnetic resonance imaging (MRI): The preferred imaging method for soft tissue sarcomas. It provides detailed images of soft tissues and helps determine the tumor's size, location, and potential spread.
- Computed tomography (CT) scan: Can be helpful to assess the tumor's extent and check for spread to the lungs.
Biopsy
A tissue sample is removed from the tumor and examined under a microscope by a pathologist to confirm the diagnosis. This is the most definitive way to diagnose a soft tissue sarcoma.
Biopsy types include:
- Fine-needle aspiration: A thin needle extracts cells from the tumor.
- Core needle biopsy: A larger needle extracts a small tissue sample.
- Surgical biopsy: Involves surgically removing part or all the tumor.
Types and stages of soft tissue sarcomas
Soft tissue sarcomas are a diverse group of cancers, with over 50 recognized subtypes. However, some types are more common than others.
Undifferentiated pleomorphic sarcoma (UPS)
Previously known as malignant fibrous histiocytoma (MFH), UPS typically develops in deep soft tissues, such as muscles, tendons, and fat. It most commonly arises in the arms and legs, but can occur in other body areas like the trunk, head, and neck. This is the most common type of soft tissue sarcoma in adults. The term "undifferentiated" means that the tumor cells don't resemble any specific type of normal tissue. It often presents as a noticeable lump that's painless in the initial stages, and can cause pain or discomfort if the tumor grows and presses on nearby nerves or muscles. Surgery is the primary treatment, often combined with radiation therapy. Chemotherapy may be used for tumors that have spread.
Rhabdomyosarcoma
Rhabdomyosarcoma is an aggressive sarcoma that arises from cells that normally develop into skeletal muscle. It most often affects children and can occur in various body parts, including the head, neck, arms, legs, and urinary and reproductive systems. Symptoms vary depending on the location but can include swelling, pain, difficulty urinating, or bowel problems. A combination of surgery, radiation, and chemotherapy is typically used. Learn more about AHN’s approach to treating rhabdomyosarcoma.
Myxofibrosarcoma
This type of cancerous tumor typically develops in the connective tissues of the arms and legs, although it can occur in other parts of the body. It is characterized by a high rate of local recurrence, meaning it has a tendency to return in the same area after being removed. While it can spread to other parts of the body, such as the lungs, this is less common.
Treatment for myxofibrosarcoma typically involves surgery to remove the tumor, sometimes followed by radiation therapy or chemotherapy. The specific treatment plan will depend on factors like the size and location of the tumor, the grade of the cancer, and the patient's overall health.
Liposarcoma
This sarcoma originates in fat cells and occurs most commonly in adults over 40. Common sites where it develops include the legs, arms, and retroperitoneum (the space behind the abdominal cavity). Often slow-growing and painless at first, these large tumors can cause swelling, pain, or functional impairments depending on their location. Surgery is the primary treatment, and radiation therapy may be recommended based on the grade and stage of the tumor. Learn more about AHN’s approach to treating liposarcoma.
Leiomyosarcoma
This is a malignant tumor arising from smooth muscle tissue, often in the uterus, stomach, intestines, or walls of blood vessels. It can develop anywhere in the body but most commonly affects the abdomen, limbs, and retroperitoneum. Symptoms vary widely depending on the tumor's size and location but may include a palpable mass, abdominal pain, bloating, weight loss, or changes in bowel or bladder habits. Surgery is the mainstay of treatment. Radiation and/or chemotherapy may be recommended as well. Learn more about AHN’s approach to treating leiomyosarcoma.
Synovial Sarcoma
Despite its name, it doesn't originate from synovial tissue but can occur near joints, often in young adults. While it can occur anywhere, synovial sarcoma is most common in the legs, particularly near the knee, and often affects young adults. Symptoms often start as a painless lump or swelling near a joint, limited range of motion, pain, and discomfort, particularly if the tumor is pressing on nerves or other tissues. Surgery, radiation therapy, and/or chemotherapy may be used alone or in combination. Learn more about AHN’s approach to treating synovial sarcoma.
Malignant Peripheral Nerve Sheath Tumor (MPNST)
MPNST is a rare sarcoma that grows from the cells that cover and protect peripheral nerves (nerves outside of the brain and spinal cord). It develops from cells surrounding nerves, sometimes linked to a genetic disorder called neurofibromatosis type 1 (NF1). This sarcoma can develop anywhere in the body where peripheral nerves are found, most often in the arms, legs, or trunk. Symptoms often present as a growing lump, pain, numbness, tingling, or weakness in the affected area. Surgery is the most common treatment, sometimes combined with radiation therapy.
Angiosarcoma
This sarcoma is an aggressive cancer that develops from the cells that line blood vessels or lymphatic vessels. Angiosarcoma can affect any part of the body but commonly occurs in the skin, soft tissues of the head and neck, breast, liver, and spleen. Symptoms will vary depending on the tumor's location. Surgery, radiation therapy, and chemotherapy may be used alone or in combination, depending on the stage and location of the cancer.
Within each type, there can be further subtypes, each with unique characteristics and prognoses. Sarcomas are also graded (from low to high) based on how abnormal the cancer cells look under a microscope. This helps predict how aggressive the tumor is likely to be. Learn more about AHN’s approach to treating angiosarcoma.
When you're diagnosed with soft tissue sarcoma, doctors use "staging" to figure out how much cancer there is and where it is in your body. This helps them plan the best treatment for you. Here are some important things to know about staging:
- Staging tells us if the cancer is only in one spot (localized) or if it has spread to other parts of your body (metastatic).
- Staging also considers the size of the main tumor (the original lump).
- The "grade" of the cancer tells us how fast the cancer cells are growing and how different they are from normal cells. A higher grade usually means the cancer is more aggressive.
- Staging also checks if the cancer is in your lymph nodes. The lymph nodes are small glands that are part of your immune system.
- Staging goes from 1 to 4. Stages 1, 2, and 3 are typically localized, while stage 4 is metastatic.
Soft tissue sarcomas treatment
Treatment for soft tissue sarcoma typically involves a multidisciplinary approach tailored to the individual patient’s needs. The specific treatment plan depends on several factors, including:
- Stage and grade: The extent of the tumor (stage) and how aggressive the cancer cells appear under a microscope (grade).
- Location and size: The tumor’s location and size can affect surgical accessibility and treatment options. Treatment decisions consider the patient's overall health, other medical conditions, and ability to tolerate certain therapies.
Surgery
Surgery is often the primary treatment for localized soft tissue sarcomas. It involves removing the tumor along with a margin of healthy tissue to reduce the risk of recurrence. Whenever possible, surgeons aim for limb-sparing surgery, preserving the affected limb's function.
Radiation Therapy
Radiation therapy uses high-energy rays to kill cancer cells. It can be given before surgery to shrink the tumor or after surgery to eliminate any remaining cancer cells. Radiation may be used as the primary treatment for inoperable or unresectable tumors.
Chemotherapy
Chemotherapy uses drugs to destroy cancer cells throughout the body. It may be given before surgery to shrink the tumor or after surgery to reduce the risk of recurrence or metastasis.In advanced cases, chemotherapy is often used to treat advanced soft tissue sarcomas that have spread to other parts of the body.
Targeted therapy
Targeted therapy drugs target specific molecules or pathways involved in cancer cell growth and survival. They are used to treat sarcomas with specific gene mutations that make them susceptible to these therapies.
Clinical trials
Clinical trials are research studies that evaluate new treatments or combinations of existing treatments. Participating in a clinical trial may provide access to promising therapies that are not yet widely available.
Follow-up care
After completing treatment, patients with soft tissue sarcoma require long-term follow-up care, including regular imaging tests and physical exams, to monitor for any signs of recurrence. Rehabilitation services, such as physical therapy or occupational therapy, may be necessary to help patients regain function and mobility after surgery or other treatments.
Soft tissue sarcomas FAQs
Questions or concerns about soft tissue sarcomas are completely normal. Your AHN care team is here to help you navigate your diagnosis and talk with you about anything that’s on your mind. If you’re feeling overwhelmed, AHN has compiled some frequently asked questions to help get you started.
Does a soft tissue mass mean cancer?
A soft tissue mass does not automatically mean cancer. Most soft tissue masses are benign (not cancerous). These masses can be caused by various factors, such as:
- Lipomas: Benign tumors made of fat cells.
- Cysts: Fluid-filled sacs.
- Hematomas: Collections of blood due to injury.
- Fibromas: Benign tumors made of connective tissue.
However, any new or growing soft tissue mass should be evaluated by a health care professional to rule out the possibility of a soft tissue sarcoma.
What is the survival rate for soft tissue sarcoma?
Survival rates for soft tissue sarcoma depend on several factors, including the stage of the cancer, the grade, the location of the tumor, the specific type of sarcoma, and the patient's overall health. The American Cancer Society relies on the Surveillance, Epidemiology, and End Results (SEER) program to help people understand cancer statistics. The five-year relative survival rates for soft tissue sarcoma for all stages is 65%.
What are the first signs of soft tissue sarcoma?
The first signs of soft tissue sarcoma are often subtle and may not cause immediate concern. The most common initial sign is a lump or swelling, which may be painless or cause mild discomfort. It might be located deep under the skin or closer to the surface. The lump might be small and easy to overlook initially.
How quickly does soft tissue sarcoma spread?
The rate at which soft tissue sarcoma spreads varies significantly depending on the grade of the tumor:
- Low-grade sarcomas (G1): These tumors tend to grow slowly and are less likely to spread to distant sites. They may remain localized for a longer period.
- High-grade sarcomas (G2, G3, G4): These tumors are more aggressive and tend to grow and spread more quickly. They have a higher risk of spreading to distant sites like the lungs.
Contact us
To make an appointment you can:
- Call (412) DOCTORS (412) 362-8677
- Schedule online
- Use MyChart and schedule with Dr. Lisa Ercolano to make an appointment
Please bring all relevant imaging on CD or through PowerShare with correlating reports for review by our team to your initial appointment.