Soft Tissue Sarcomas

Symptoms and risk factors

As soft tissue sarcoma grows, you may notice a painless lump.

A sarcoma may cause a painless lump under the skin (usually on the arms, legs, or trunk), but often no other symptoms exist at first. As the sarcomas grow and press on organs, nerves, muscles, or blood vessels, they can cause:

• Pain
• Breathing trouble
• Abdominal swelling (or distension)

These symptoms can indicate a variety of different problems. Make sure to discuss any that you experience with your doctor, especially if you also have risk factors for soft tissue sarcoma.

Certain medical conditions can increase your odds of developing this cancer.

Soft tissue sarcoma is not a common type of cancer—it accounts for about 6,000 cases every year. While we don’t know what causes most soft tissue tumors, risk factors include:

• Inherited disorders, such as:
  • Retinoblastoma
  • Neurofibromatosis (von Recklinghausen disease)
  • Tuberous sclerosis (Bourneville disease)
  • Gardner syndrome
  • Li-Fraumeni syndrome
  • Werner syndrome
  • Gorlin syndrome (Nevoid basal cell carcinoma syndrome)
• Past radiation therapy for certain cancers
• Chronic swelling in the arms or legs (tied to some lymph vessel cancers in adults)
• Extensive exposure to certain chemicals, including Thorotrast, vinyl chloride, or arsenic

Screening and prevention

Tell your doctor if you notice any unusual lumps on your body.

Sarcomas are very rare, and we don’t yet have a specific prevention strategy for this type of cancer, but research is ongoing. Many times, people find out they have cancer incidentally after undergoing imaging for a soft tissue injury.

Like other cancers, early detection can make these cancers easier to treat. Although there is no general screening method, talk to your doctor if you notice an unusual lump or soft tissue sarcoma symptoms. They can monitor it or recommend tests to determine the cause, whether it's cancer or something else.

Diagnosis

Our interventional radiologists are experts in performing minimally invasive biopsies in collaboration with your surgeon.

Successful treatment depends on a complete and accurate diagnosis. Each of our patients receive a thorough evaluation so we can create an individualized plan. To diagnose sarcoma, we may conduct the following:

• Medical history: We will discuss your symptoms and medical history, including whether you have any risk factors.
• Physical examination: We will examine you thoroughly, including any lumps you have.
• Blood work
• Imaging scans: A variety of imaging scans can help identify the presence, type, and size cancer, including:

  • MRI: This imaging test uses high-powered magnets to create a picture of the affected area.

  • CT (CAT) scan: This imaging test uses X-ray technology and computers to create a detailed picture of your affected tissues.

  • PET scan: For this imaging test, your doctor injects radioactive glucose. The PET scan follows the glucose's path, which identifies the presence of cancerous cells.

• Biopsy: Your surgical oncologist will determine whether you need a minimally invasive procedure to collect a biopsy, or tissue sample, using imaging guidance. If you do, a specially-trained interventional radiologist and your surgeon will decide on the appropriate path to remove tissue from the affected area. This collaboration is important for ensuring the biopsy doesn't interfere with the surgical plan, and not every cancer center is equipped to perform biopsies using this approach. Once the tissue is removed, a pathologist will examine it closely under a microscope. This will help us diagnose the cancer and understand how quickly the cells are spreading.

Types of cancer

There are more than 80 different types of soft tissue sarcoma

This disease is one of the most rare cancers, but different subtypes are more common in certain body parts and affect specific age groups. Therefore, each type of soft tissue cancer requires a different approach to treatment. Some of the more well-known subtypes include:

• Alveolar Soft Part Sarcoma are rare, slow-growing tumors that mainly affect teens. They often grow deep in the thigh, though they can also show up in the heads and necks of younger children.
• Clear Cell Sarcoma mainly affects younger adults ages 20 to 40, growing just beneath the skin and affecting tendons and muscle sheaths—usually in the foot and ankle.
• Epithelioid Sarcoma mainly develops in the forearm or hand, most frequently in male patients in their teens or 20s.
• Fibrosarcoma develops from the tissues keeping bones, organs, and muscles secure. One type of the disease mainly affects middle-aged adults, usually in the legs, arms, or trunk. The other type affects children—most frequently at birth and seldom past age two. Outcomes are better for children, with a lower chance the cancer will spread or return.

• Gastrointestinal Stromal Tumor (GIST) is a rare tumor that tends to affect adults. It develops in the abdomen, where it can grow quite large before it’s noticed. This can make it harder to remove.

• Leiomyosarcoma develops from blood vessels in the trunk and limbs and mainly affects adults starting in middle age.
• Liposarcoma develops from fatty tissues and usually appears in the arms, legs, abdomen, or buttocks—most frequently in men and women over 50.
• Rhabdomyosarcoma is made of cells that typically develop into the skeletal muscles we use to control our body. These tumors are found mainly in the head and neck, urinary and reproductive organs, arms and legs, and trunk. While the tumors can affect all ages, they’re mainly found in children under five and are the most common childhood soft tissue sarcoma. They can grow quite large in a short time.
• Synovial Sarcoma can develop deep in the muscles of the arms and legs and mainly affect young adults, particularly men in their 30s.

Treatments

Treatment will depend on the type, size, and location of disease, as well as your goals and preferences.

We offer the area's most advanced and comprehensive care. Our team of sub-specialized physicians and advanced practice clinicians have years of experience treating people with these types of cancer. You'll also benefit from our multidisciplinary approach, which allows us to collaborate with experts in a variety of fields, including:

• Fellowship-trained surgical and orthopedic oncologists
• Medical oncologists
• Radiation oncologists
• Interventional radiologists
• Pathologists
• Reconstructive surgeons
• And others

In addition, we use the most cutting-edge research to guide your care. That means you can access the latest cancer clinical trials and the most innovative ways to treat your disease.

Our team of specialists discusses each case in bi-weekly tumor boards, where we can collectively recommend the best treatment recommendation. Children and teens may respond to treatment differently than adults. Therefore, we’re working to safely lower the amount of radiation and chemotherapy younger patients receive while still providing successful cancer treatment.

Surgery

Patients with this type of sarcoma are often referred to a surgeon for evaluation. Because surgery is often the best way to remove these types of cancer, most patients will undergo a "wide resection," during which the surgeon will remove the tumor and a layer of surrounding healthy tissue to minimize the risk of recurrence. When sarcoma impacts the arms or legs, we are experienced in performing limb-sparing surgery, which eliminates the need for amputation in more than 95 percent of patients. When amputation provides the best chance for cure, we offer comprehensive services to help our patients physically and emotionally cope with losing a limb.

Your surgical team will plan your surgery based on:

• The location and size of your tumor
• What other internal structures are involved
• What we can reasonably and safely save
• How we can reconstruct what is left to ensure maximum function

These sarcomas are often large and/or complex to operate on because of nearby blood vessels, nerves, and muscles. That's why it's important to choose a surgeon with extensive experience in treating sarcomas in collaboration with other specialists. Our orthopedic oncologists and surgeons have decades of combined experience, making us highly specialized. We also work closely with other specialists to determine the right approach for you. For example, we may collaborate with reconstructive surgeons to help rebuild internal structures or to restore the body's appearance. Your care team will also include radiation and medical oncologists who may recommend radiation therapy and/or chemotherapy before or after surgery.

Recovery and follow-up after surgery

Often, we can perform surgery as an outpatient procedure, allowing patients to go home the same day. We take an innovative approach to managing postoperative pain, using local anesthetics and regional blocks, which helps you get moving earlier. The length of recovery will vary based on the location of your surgery and other factors. On average, you can expect to have some discomfort and swelling for several weeks. Physical therapy and rehabilitation will play an important role in helping you to regain strength and mobility.

Soft tissue sarcoma can recur, or reappear, even after treatment. We will work with you to ensure we catch any recurrence as early as possible. Factors that can affect recurrences include:

• Sarcoma size: A large sarcoma size has a higher chance of recurring.
• Sarcoma grade: Low-grade sarcomas are less likely to recur.
• Location of sarcoma: Sarcomas that affect the inside of the abdomen and the organs around it are more likely to recur than those that affect the limbs.
• How much of the sarcoma was surgically removed: If your surgical oncologist was unable to completely remove the sarcoma, you may be more likely to have a recurrence. Sometimes the location of the sarcoma does not allow for complete removal. In those cases, we will follow up surgery with radiotherapy.
• Previous recurrence: A sarcoma that has already recurred is more likely to recur again
• Type of sarcoma histology: The specific details of the tissue that make up the sarcoma can affect recurrence.
• Your age: If you are older, it is more likely for the sarcoma to recur.

Chemotherapy

This treatment can destroy some but not all types of soft tissue sarcomas. It uses powerful drugs delivered either orally or through your veins (IV). Our medical oncologists specialize in treating sarcomas with these drugs, including chemotherapies available through clinical trials. They'll work with your other specialists to determine if and/or when chemotherapy will offer you the best outcome while minimizing side effects. Sometimes, chemotherapy may be given with or without radiation before surgery to help shrink the tumor and increase the chances of a limb-sparing procedure.

Chemotherapy may also be used to treat recurrent or metastatic soft tissue sarcoma.

Learn More About Chemotherapy

Immunotherapy and targeted therapy

Targeted therapies or agents are newer drugs that offer hope for some subtypes of this cancer. These medications can "target" genetic changes in cancerous tumors, which allows us to further personalize your treatment. For example, the targeted therapy imatinib (Gleevec) is already approved for treating gastrointestinal stromal tumors (GIST). Imatinib is typically recommended for a year after surgery to ensure the cancer does not return. It’s also used when tumors are too large for surgery, in an attempt to shrink them and make them operable.

Similarly, immunotherapy drugs help to fight cancerous cells in specific sarcomas using the body's immune system. We've been leaders in using this type of therapy to effectively treat cancers and improve the quality of life for our patients. We continue investigating new targeted therapies and immunotherapies through clinical trials. We may recommend one of these clinical trials if we think it could help to improve your outcome or quality of life.

Learn More About Targeted Therapy

Learn More About Immunotherapy

Radiation therapy

Many patients benefit from radiation therapy before surgery (neoadjuvant therapy) to shrink the tumor or after surgery to minimize recurrence. This decision is made by your multidisciplinary team based on the size and location of your tumor and other factors. When radiation therapy is recommended for sarcoma, we use the latest advances to administer it in a way that limits radiation exposure to healthy tissue. And, we regularly monitor how your body responds to treatment to ensure we address and minimize side effects.

Most commonly, radiation is delivered daily over five to six weeks using intensity-modulated radiation therapy (IMRT). This innovative form allows our radiation oncologists to change treatment intensity as they go based on tissue type, delivering specific doses to different parts of a tumor, and sparing healthy tissue. This is particularly useful for tumors in hard-to-treat areas, such as the spine or pelvis.

Others may benefit from proton therapy, which is an exceptionally precise way to administer radiation to the exact size and shape of the tumor. Many patients report fewer side effects and a better quality of life, and this is often the best, safest way for pediatric patients to undergo radiation. If a soft tissue sarcoma has spread to the lungs or other critical organs, we may use stereotactic body radiation therapy (SBRT) to deliver radiation to cancer cells.

Learn More About Radiation Therapy

Why choose us

We treat a high number of patients with sarcoma with exceptional outcomes. Rare tumors require specialized care from doctors who have experience treating it using the latest advances in surgery, radiation, and medicine. In fact, research proves that clinical outcomes are better at cancer centers who see a high number of patients with a particular disease. Our sarcoma doctors know how to treat soft tissue sarcoma because they have additional subspecialty training and focus exclusively on caring for people with these types of cancers. We see hundreds of patients every year, making us one of the most experienced in the country.

We personalize your cancer treatment using the latest therapies and approaches, including those only available in clinical trials. Compared to other cancer types, there are often fewer treatment options for sarcomas because they are so rare. However, our sarcoma specialists stay up-to-date on the most advanced treatment options, including new drugs and treatments that are hard to find elsewhere. We regularly investigate innovative immunotherapies, targeted therapies, and other treatments that offer hope for our patients.

You'll benefit from a team of healthcare providers with both compassion and decades of combined experience. From the beginning of your care, we involve healthcare providers who specialize in different aspects of cancer-related treatment. Through regular tumor boards, we discuss recommendations that consider your treatment from every angle. This allows us to effectively work together to plan, personalize, and coordinate treatment that will work best for you. Unlike other cancer centers, you won't have to travel to meet with different specialists to get all of the information you need. You'll receive the best possible recommendation with input from all of the right experts, under one roof.

Support services

In addition to your medical providers, your care team may include social workers, case managers, dietitians, rehabilitation specialists, genetic counselors, and others who can help to address any psychological and practical needs you have. Our goal is to support you and your family as you navigate the impact of a cancer diagnosis on your emotional health and day-to-day life.

Learn More About Cancer Support Services

Genetic counseling

Because some inherited conditions can affect your risk of developing these cancers, you may benefit from meeting with one of our board-certified genetic counselors. Our genetic counseling services can help patients and their family members understand and manage their cancer risks through screening, surveillance, and other strategies.

Learn More About Genetic Counseling

Personalized rehabilitation

Cancer treatments can negatively affect your quality of life, and surgery can affect your mobility. Through comprehensive and coordinated rehabilitation, our physical and occupational therapy experts can help you achieve your greatest level of independence and function wherever you are in your journey back to health.

Learn More About Cancer Rehabilitation

Survivorship

For every patient who comes through our clinic, we create a personalized survivorship plan designed to address all of their physical, psychological, and social needs. Based on the treatments they received, we'll tailor recommendations for follow-up care. This may include connections to other experts throughout MedStar Health, including doctors in internal medicine, gastroenterology, cardiology, and other specialties.

Learn More About the Cancer Survivorship Program