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What Is Soft Tissue Sarcoma?
Soft tissues play a crucial role in the body by connecting, supporting, and surrounding organs and structures. Soft tissue sarcomas are a type of cancer that can develop in any soft tissue of the body. They mainly occur in the extremities (arms and legs) and trunk (chest and abdomen).
These cancers can affect people of all ages.
There are many types of soft tissue sarcomas. The cells of these sarcomas look different under a microscope based on the type of soft tissue in which the cancer began growing.
What are the types of soft tissue sarcomas?
Soft tissue sarcomas fall within a spectrum of cancers, including:
- Blood and lymph vessel tumors.
- Fibrous tissue tumors affecting connective tissues, such as tendons.
- Fibrohistiocytic tumors affecting deep tissues or bones.
- Fat tissue tumors.
- Peripheral nervous system tumors.
- Smooth muscle tumors.
The most common soft tissue sarcomas in children are rhabdomyosarcomas (RMSs) and non-rhabdomyosarcoma soft tissue sarcomas (NRSTSs).
Rhabdomyosarcomas
Rhabdomyosardoma (RMS) usually begins in muscles that attach to the body's movement bones. However, it can begin in other places.
There are several types of rhabdomyosarcoma. These have historically been classified by pathologic type, including alveolar, embryonal, pleomorphic, and spindle cell. Recently, these types of tumors have started to be classified by genetic fusion panels.
Non-rhabdomyosarcoma soft tissue sarcomas
Non-RMS soft tissue sarcomas (NRSTSs) are more common in adolescents and young adults. Infants can also get NRSTSs, but their tumors are distinctive from infantile fibrosarcoma and malignant (cancerous) hemangiopericytoma — two conditions not seen in adolescents.
There are over 100 types of NRSTSs. The most common types in children include:
- Infantile fibrosarcoma — Can present in a variety of sites on the body. They can now be targeted with a new medication targeting a particular gene fusion if it's present.
- Malignant peripheral nerve sheath tumors (MPNSTs) — Affect nerves.
- Synovial sarcomas — Affect the muscles and tendons, especially near joints.
- Undifferentiated sarcomas — Affect soft tissues anywhere in the body, mostly the arms or legs.
There are also tumors with multiple tissue types and tumors of unknown tissue origin.
How common are soft tissue sarcomas?
Soft tissue sarcomas are relatively rare, affecting up to 900 children per year in the United States. Of these sarcoma cases, more than a third are rhabdomyosarcoma (RMS). RMS is the most common soft tissue sarcoma in children ages 14 and under.
What causes soft tissue sarcoma?
Childhood soft tissue sarcoma occurs from certain changes in how soft tissue cells function, especially how they grow and divide into new cells. Often, the exact cause of these changes is unknown.
Does soft tissue sarcoma spread quickly?
Soft tissue sarcoma can spread quickly. According to the National Cancer Institute (NCI), about one-third of children with soft tissue sarcomas experience cancer metastasis. Metastasis occurs when cancer spreads to other parts of the body.
Soft tissue sarcoma risk factors
A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop a soft tissue sarcoma. And soft tissue sarcoma will develop in some children who don't have a known risk factor.
Certain diseases and disorders increase the risk of developing childhood soft tissue sarcomas, including:
- Acquired immune deficiency syndrome (AIDS).
- Adenosine deaminase-deficient severe combined immunodeficiency.
- Beckwith-Wiedemann syndrome.
- Costello syndrome.
- Dicer1 syndrome.
- Li-Fraumeni syndrome.
- Epstein-Barr virus.
- Neurofibromatosis type 1 (NF1).
- Noonan syndrome.
- Previous radiation treatment.
- RB1 gene changes.
- Tuberous sclerosis.
- Werner syndrome.
Soft tissue sarcoma complications
Without treatment, soft tissue sarcoma will continue to grow and become more dangerous. Over time, it can cause pain and spread to other parts of the body, especially the lungs, which can be life‑threatening.
As the tumor grows, it may press on nearby nerves and muscles. This can lead to numbness, weakness, and trouble using the affected body part.
If the cancer spreads or becomes too large, it may be much harder — or even impossible — to remove with surgery. This can result in serious organ damage or even amputation. Survival rates drop sharply once the cancer reaches advanced stages.
What is the life expectancy of someone with soft tissue sarcoma?
The life expectancy and outcome of treatment depend on the type, size, location, and biology of a soft tissue sarcoma. Many children, especially with low-grade, small tumors, have excellent long-term survival. The ability to perform a complete surgery also improves long-term survival.
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What Are the Signs and Symptoms of Soft Tissue Sarcoma?
Soft tissue sarcomas often appear on the arms, legs, chest, or belly. If in the extremity or on the trunk, it often will appear as a painless lump under the skin that doesn’t go away. The lump may be the only symptom at first.
As sarcomas grow, they put pressure on surrounding tissues and organs, causing other symptoms.
Common symptoms of sarcoma may include:
- Bleeding in the nose, throat, vagina, or rectum.
- Blood in the urine.
- Bulging or crossed eyes.
- Headache.
- Pain in the area of the lump.
- Swelling in the area near the lump.
- Trouble peeing or pooping.
- Weakness in the affected area.
Rarely, sarcomas can cause other symptoms, including:
- Fever.
- High blood sugar.
- Low blood sugar.
- Night sweats.
- Weight loss.
When should I see a doctor about my child's soft tissue sarcoma symptoms?
You should have your child's provider check any growing lump or unexplained mass that lasts more than a few weeks as soon as possible. Early treatment greatly improves outcomes.
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How Do You Diagnose Soft Tissue Sarcoma?
To diagnose soft tissue sarcoma, your child’s provider will perform a physical examination. They'll ask when your child’s symptoms started and the frequency of symptoms. They'll also ask about your child’s and your family’s personal and health history. They may order tests to help identify the type of sarcoma.
Tests to diagnose soft tissue sarcoma
Your child's provider may order several different tests to diagnose soft tissue sarcoma. These include:
- Bone marrow aspiration and biopsy — Involves inserting a hollow needle into the hipbone to remove bone marrow, blood, and a tiny piece of bone for testing. Providers remove samples from both hip bones. A pathologist will view the samples under a microscope to check for cancer.
- CT scan — May include an injected dye that goes into your child’s vein to make it easier to see the tumor in imaging.
- Lumbar puncture — Involves placing a hollow needle between two bones of the spine to collect cerebrospinal fluid (CSF) around the spinal cord. The CSF sample is reviewed under a microscope for the presence of cancer cells. Also called an LP or spinal tap.
- MRI — Uses large magnets and radio waves to help see soft tissues more clearly.
- PET scans — Involves injecting a small amount of radioactive glucose (sugar) through an IV. The PET scanner detects areas where glucose is being used in the body. Cancer cells show up brightly in the scans because they take up more glucose than normal cells.
- Sentinel lymph node biopsy — A sentinel lymph node is the first lymph node where cancer will likely spread. In a sentinel lymph node biopsy, providers remove the sentinel lymph node and check it for cancer.
- Tissue biopsy — Involves removing a tumor tissue sample and sending it to a lab for testing. Providers may take biopsies from primary tumors, lymph nodes, and/or any surrounding areas affected.
- Ultrasound — Can help determine the location and size of the tumor.
- X-ray — May help determine the location of the tumor in the chest or abdomen.
Your child’s doctor may order other, more specialized tests depending on their symptoms and the cancer's possible location. The results of these tests will help your child's provider plan treatment.
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How Do You Treat Soft Tissue Sarcoma?
The goal of soft tissue sarcoma treatment is to cure the cancer or get rid of it as much as possible.
Surgery for soft tissue sarcoma
Surgery to remove the tumor is the main treatment for children with soft tissue sarcomas.
The following types of surgery can treat most soft tissue sarcomas:
- Wide local excision — The surgeon removes the tumor with a “margin.” The margin of normal tissue around it ensures that there is no tumor left behind.
- Amputation — Removes all or part of the arm or leg with cancer. Care teams avoid amputation when possible, but it may be the best or only option in some cases. The preferred approach is to perform a wide local incision, preserving the extremity while getting an adequate removal of the tumor.
- Second look surgery — Can remove any remaining cancer cells or check cancer margins around the tumor removal site. This surgery is most often needed if the lesion was removed before it was known to be a soft tissue sarcoma.
Chemotherapy
If your child has rhabdomyosarcoma, they may receive with neoadjuvant chemotherapy before surgery. This can shrink tumors so that surgeons need to remove less surrounding tissue. Adjuvant chemotherapy after surgery can kill any remaining cancer cells.
For NRSTSs, chemotherapy is used only for high-grade or inoperable tumors.
Radiation
Radiation therapy uses high-energy x-rays or other types of radiation to kill cancer cells or prevent their growth. It can treat some cases of RMS as neoadjuvant or adjuvant therapy combined with surgery.
Immunotherapy
Immunotherapy is a treatment that uses a child’s own immune system to fight cancer. It can treat some types of soft tissue sarcoma.
Targeted therapies
Targeted therapies use drugs and other substances to block the growth and spread of specific enzymes, proteins, and other molecules that spur cancer development.
Clinical trials
For some children with soft tissue sarcomas, participation in a clinical trial may be the best treatment option. Clinical trials are part of the research process to:
- Find new cancer treatments.
- Determine their safety.
- Compare them with standard treatments.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients participating in clinical trials may receive the standard treatment or be among the first to receive a new treatment.
How effective is treatment?
Many factors affect the recovery rate and treatment options:
- Age of patient — The outcome is usually more favorable when discovered and treated in childhood.
- Size and location of tumor — The location and size of the tumor and how deep it is into the soft tissue will affect recovery./li>
- Time of diagnosis — Tumors that are found earlier often can be easier to remove and respond better to treatment.
- Type of tumor — Certain types of sarcomas have better recovery rates.
While treatment can cure soft tissue sarcoma, the cancer can also recur (come back).
How long does it take to recover after treatment for soft tissue sarcoma?
Recovery depends on the type of treatment and rehabilitation needed. For example, your child may need physical therapy after surgery. Your child's care team will go over their recovery plan with you.
Your child will also need long-term follow-up care to manage their cancer or prevent it from coming back.
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Why Choose UPMC for Soft Tissue Sarcoma Care?
At UPMC Children’s, every child with soft tissue sarcoma receives an individualized treatment plan and family-centered care. You can expect:
- Expert cancer care — Soft tissue sarcoma can be complex; you want to choose a provider with vast experience in treating this condition. Our surgeons have trained at the nation's leading cancer treatment centers. They have extensive experience in soft tissue sarcoma surgery.
- Access to advanced care — Cutting-edge research and the latest technology ensure our patients' best possible outcomes.
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