The American Cancer Society estimates about 13,590 new cases of soft tissue sarcoma (STS) will be diagnosed in the United States in 2024, and about 5,200 of those cases are expected to be fatal.

Sarcoma is a family of rare cancers that starts in the body’s connective tissue, including muscles, bones, tendons, cartilage, nerves, fat, and blood vessels.

In adults, sarcomas aren’t common, making up only about 1% of cancer diagnoses. Still, about 15% of childhood cancers are sarcomas.

Although less common than other forms of cancer, a sarcoma cancer diagnosis can have a significant impact on the lives of those affected and their loved ones.

Sarcoma can start anywhere in the body, and there are more than 50 distinct types, each with its own characteristics and treatment approaches.

Many people are unfamiliar with sarcoma cancers, which can lead to delays in seeking diagnosis and treatment. The cause of sarcoma is often unknown, but age, genetics, and prior radiation treatment may play a role in the disease.

Some risk factors can increase your chance of getting it, including a history of certain diseases.

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A Multidisciplinary Approach to Sarcoma Care

Once sarcoma is at a more advanced stage, it can be harder to treat.

The good news is UPMC Hillman Cancer Center‘s team of orthopaedic surgeons and medical and radiation oncologists offer a comprehensive approach to sarcoma treatment. They partner with pathologists, pain management specialists, and a range of other experts to find the right sarcoma treatment plan for you.

As the only National Cancer Institute-designated Comprehensive Cancer Center in the region, UPMC Hillman Cancer Center gives those diagnosed with sarcoma early access to cutting-edge treatments and clinical trials.

UPMC Hillman specialists treat a full range of common and rare sarcomas, including:

  • Bone and soft tissue sarcoma — Cancers that form in bone or soft tissues like blood vessels, muscle, or fat.
  • Ewing sarcoma — A childhood cancer that forms in bone and soft tissues.
  • Kaposi’s sarcoma — A cancer that forms in the skin, mucus membranes, lymph nodes, skin, and other organs.
  • Leiomyosarcoma — A cancer that forms in the body’s smooth muscle cells.

To achieve the best results and help prevent the cancer from returning, UPMC experts offer state-of-the-art sarcoma care, including:

  • Chemotherapy before or after surgery or to treat cancer that has spread to other organs.
  • Imaging tests and biopsies to diagnose sarcoma.
  • Staging to design the best treatment plan. Stage I is the least advanced, and Stage IV is the most advanced.
  • The latest surgical techniques to treat bone cancers.
  • Targeted therapies where small molecules block cancer cells’ pathways to survive and multiply.
  • Behavioral and palliative care, nutritional guidance, and more.

Sarcoma Symptoms and Diagnosis

Sarcoma symptoms vary based on the size of the tumor, its progression, and where it’s located. For example, a sarcoma on or near the heart can cause chest pain, shortness of breath, and fatigue.

Bone and soft tissue cancers may cause symptoms like swelling, pain, stiffness, weight loss, and fractures.

A proper diagnosis is key to successfully treating bone and soft tissue cancers. State-of-the-art tools and techniques to diagnose and stage sarcoma at UPMC include:

  • Scans to help your doctor diagnose bone or soft tissue cancer, such as CT scans, MRIs, and radiographic tests.
  • Biopsies, such as a minimally invasive core needle biopsy and, in certain cases, an excisional biopsy with general anesthesia.

Staging to Develop Treatment

Staging allows doctors to determine the exact type, size, location, and spread of sarcoma cancers to develop the best treatment plan.

A care team will conduct tests and procedures to classify the type and stage of your sarcoma using four factors based on the American Joint Committee on Cancer system:

  • Tumor: Its size and location.
  • Nodes: Whether the cancer has spread to lymph nodes.
  • Metastasis: Whether the cancer has spread to distant organs.
  • Grade: How abnormal the cancer cells look under a microscope.

Each factor gets a stage number and is added to assign an overall stage to the cancer. Stage I sarcoma is the least advanced, and Stage IV is the most advanced.

Sarcoma Care and Treatment Options to Consider

Once your sarcoma is diagnosed and staged, an orthopaedic surgeon or orthopaedic oncologic surgeon will likely refer you to a multidisciplinary cancer care team.

This team will evaluate your cancer, review your diagnostic tests, and meet to discuss your treatment plan.

Sarcoma treatments depend on your age, cancer stage, and tumor type.

Your treatment plan may involve a combination of chemotherapy, radiation, and surgery. In certain sarcomas, chemo and radiation can help shrink the tumor before surgery or help prevent it from spreading after surgery.

Radiation for Sarcoma

The latest sarcoma drugs and treatments include newer systems that provide a targeted dose of radiation to spare healthy tissue near the sarcoma. Sarcoma radiation therapies offered at UPMC include:

  • 3D radiotherapy: This uses targeting data to focus on the tumor and avoid the healthy tissue around it. This targeting allows for higher levels of radiation in treatment, which is better for shrinking and killing tumors.
  • Intensity-modulated radiation therapy (IMRT): This gives radiation therapists the ability to “sculpt” the edges of a tumor, minimizing the damage to nearby healthy tissue. It allows for more precision and accuracy, resulting in the potential for fewer side effects and higher cure rates.
  • Image-guided radiation therapy (IGRT): This uses advanced imaging to track changes through all stages of cancer treatment and adjust your treatment plan as needed.
  • Stereotactic radiosurgery (SRS): This doesn’t involve actual surgery, but instead pinpoint-focused radiation beams are used to treat cancer in specific areas with surgical excision.
  • High-dose rate (HDR) brachytherapy: Cancer cells left after surgeons remove the tumor can form new tumors. That’s why doctors may use high-dose rate (HDR) brachytherapy at the surgical site to prevent tumor recurrence. This helps prevent tumor regrowth and reduces injury to healthy tissue.

Surgery for Sarcoma

For smaller soft tissue sarcomas that haven’t spread, surgery to remove the tumor is often the first step.

Doctors will prioritize minimally invasive methods to remove tumors using the smallest incisions possible. This technique removes sarcomas and the least amount of healthy tissue possible for quicker recovery and less pain.

While most people benefit from these less-invasive approaches, some tumors are too large or difficult to reach. In cases that require open surgery, surgeons make sure to keep incisions minimal.

This may involve using highly specialized technologies like regional therapy for treating sarcoma and newer sarcoma treatments, including:

  • Isolated hepatic perfusion: This treats metastatic cancers that have spread to the liver. Surgeons separate the blood supply of the liver from the circulatory system and disperse chemo solution through the liver.
  • Laparoscopic surgeries for metastatic disease: This surgical technique uses a long, thin instrument with a tiny camera to make small incisions. It may be appropriate for those with certain soft tissue sarcomas.
  • Limb and pelvic reconstruction.

About UPMC Hillman Cancer Center

When you are facing cancer, you need the best care possible. UPMC Hillman Cancer Center provides world-class cancer care, from diagnosis to treatment, to help you in your cancer battle. We are the only comprehensive cancer center in our region, as designated by the National Cancer Institute. We have more than 70 locations throughout Pennsylvania, Ohio, and New York, with more than 200 oncologists – making it easier for you to find world-class care close to home. Our internationally renowned research team is striving to find new advances in prevention, detection, and treatment. Most of all, we are here for you. Our patient-first approach aims to provide you and your loved ones the care and support you need. To find a provider near you, visit our website.