Medically Reviewed |
Last reviewed by
Gohar S. Manzar, M.D., Ph.D.,
J. Andrew Livingston, M.D., M.S. and
Valerae O. Lewis, M.D.
on
March 23, 2026
Soft tissue sarcomas are a group of cancers that begin in areas that surround, connect or support the body’s structure or organs.
This includes fat, cartilage, connective tissue, muscles, skin, nerves, tendons, joints, blood vessels and lymph vessels.
The most common locations of soft tissue sarcomas are the arms and legs, but they can also grow in the head and neck area, chest, abdomen and pelvis. Soft tissue sarcoma can occur in children and adults. This information focuses on soft tissue sarcoma in children.
Types of childhood soft tissue sarcoma
Rhabdomyosarcoma (RMS) is the most common childhood soft tissue sarcoma, with a few hundred cases diagnosed in the United States each year. It grows in muscles attached to the skeleton.
Other childhood soft tissue tumors are sometimes called non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). Together, this group of different cancers makes up about 4% of pediatric cancers. They are classified by the part of the body in which they start and by the type of cells they contain.
Other than rhabdomyosarcoma, the most common soft tissue sarcomas in children include:
- Fat tissue tumors: Liposarcoma in children is usually low or intermediate grade and grows slowly. The most common type is myxoid liposarcoma.
- Connective tissue tumors:
- Desmoid tumors (aggressive fibromatosis), which usually grow slowly and are unlikely to spread. Children with familial adenomatous polyposis (FAP) are at higher risk.
- Fibrosarcoma often occurs in children under age 4, especially infants. Tumors tend to grow quickly but usually do not spread.
- Muscle tumors other than RMS: Leiomyosarcoma is associated with Epstein-Barr virus in children with HIV/AIDS. Survivors of retinoblastoma are also at higher risk.
- Skin tumors:
- Plexiform fibrohistiocytic tumors begin as painless masses on or under the skin, usually in the arm, hand, or wrist.
- Undifferentiated pleomorphic sarcoma can develop in children who have had radiation therapy or retinoblastoma.
- Nerve tumors: Malignant peripheral nerve sheath tumors grow in the covering of nerves outside the brain and spinal cord. Children with neurofibromatosis type 1 (NF1) are at higher risk.
- Tumors of unknown origin: Synovial sarcoma can develop in different parts of the body, most often near joints in the arms or legs, but also in the head, neck or trunk. Despite its name, it does not arise from synovial (joint) tissue. Instead, it is called “synovial” sarcoma because, under a microscope, the tumor cells can resemble cells found in joint linings. However, the exact type of normal cell these tumors come from is still unknown.
- Blood vessel tumors:
- Angiosarcomas, which grow quickly in lymph or blood vessels.
- Epithelioid hemangioendotheliomas, which usually develop in the liver in infants and may grow slowly.
Who gets childhood soft tissue sarcoma
Soft tissue sarcoma can occur at any age, but certain groups are at higher risk, including:
- Teenagers and infants under 1 year of age
- People with certain genetic conditions, such as neurofibromatosis type 1 (NF1), Li-Fraumeni syndrome, inherited retinoblastoma, Werner syndrome, familial adenomatous polyposis (FAP)
- Individuals with HIV/AIDS or Epstein-Barr virus
Having a risk factor does not mean a child will develop soft tissue sarcoma. However, it is important to discuss family and personal medical history with your child’s doctor. Genetic counseling and testing may be recommended.
Childhood soft tissue sarcoma symptoms
Soft tissue sarcoma often presents as a firm painless lump that steadily increases in size and may be fixed in place. Other symptoms vary depending on the type and location of the tumor.
They may include:
- Pain or tenderness, sometimes worsening at night
- Fatigue
- Difficulty moving a limb if near a joint
- Unexplained fever or weight loss
These symptoms do not always indicate soft tissue sarcoma, but they should be evaluated by a doctor.
Childhood soft tissue sarcoma diagnosis
A thorough work-up is essential for successful treatment. Tests may include:
- Imaging: CT scans, MRI scans, PET scans, X-rays and bone scans may be used to assess the tumor’s location and determine whether it has spread.
- Biopsy: A biopsy is the only way to confirm that a tumor is cancer. It may be performed using a needle or through an open surgical procedure.
- Bone marrow aspiration or biopsy and blood tests: These tests may be done in selected cases.
- Genetic testing: Genetic testing may be recommended if there is a suspicion of inherited cancer risk.
Childhood soft tissue sarcoma treatment
Treatment is tailored to each child and may include:
- Surgery: Removal of the tumor while sparing surrounding healthy tissue. Limb-sparing procedures are often possible.
- Chemotherapy: Often the first step to treat micrometastases not visible on imaging and to treat or shrink the primary tumor prior to surgery and/or radiation.
- Radiation therapy: Used to destroy remaining cancer cells or when surgery is not possible. Advanced techniques minimize the impact on growing bodies.
- Targeted therapy: Innovative agents designed to treat cancer based on its genetic or molecular profile.
- Clinical trials: Access to national and international research protocols may be recommended.
Childhood soft tissue sarcoma survival rates
Every child’s situation is unique, and outcomes can vary based on factors like the type of tumor, where it is located, how advanced it is at diagnosis and your child’s age.
Some types, such as rhabdomyosarcoma, often respond well to treatment, especially when found early and cared for by an experienced team. Other soft tissue sarcomas can behave differently, and outcomes may vary depending on the specific tumor type and stage.
Because there are so many factors involved, your child’s care team is the best source of information about what to expect. They can help you understand your child’s specific diagnosis, treatment plan and outlook, and answer any questions along the way.
Living with childhood soft tissue sarcoma
Supportive care is essential for recovery and quality of life:
- Physical therapy and rehabilitation after surgery
- Psychosocial support for children and families
- Regular follow-up to monitor for recurrence or long-term treatment effects
- Coordination of care among pediatric oncology specialists
Risk factors
A risk factor increases the chance of developing a certain disease. Risk factors for childhood soft tissue sarcomas include:
- Inherited genetic conditions: NF1, Li-Fraumeni syndrome, inherited retinoblastoma, Werner syndrome
- Familial adenomatous polyposis (FAP)
- Viral infections: HIV/AIDS and Epstein-Barr virus
- Age: Teenagers and infants under 1 year of age
Are soft tissue sarcomas hereditary?
Some soft tissue tumors can be inherited through passed-down genetic mutations. This accounts for approximately 20% of cases. Genetic counseling may be recommended. Learn more about the risk to you and your family on our genetic testing page.
Learn more about childhood soft tissue sarcoma:
- Childhood soft tissue sarcoma symptoms
- Childhood soft tissue sarcoma diagnosis
- Childhood soft tissue sarcoma treatment
Rare stage 4 sarcoma survivor: ‘Thanks to UT MD Anderson, I’m already beating the odds’
In May 2014, I was a fresh college graduate in New York City who was excited to begin my life as a professional ballerina.
A few weeks later, I was in the hospital with a broken sacrum and a traumatic brain injury, due to a terrible car accident.
Once I recovered, it was clear that I couldn’t dance professionally. So, I did a career pivot and applied to medical school, which had always interested me. About halfway through it, I developed severe preeclampsia, a complication of pregnancy that causes dangerously high blood pressure. I ended up needing an urgent C-section at 37 weeks, followed by an immediate hysterectomy to stop a massive hemorrhage and save my life.
After those two experiences, I really thought I’d reached my quota for brushes with death. Then, just a week after graduating from medical school last May, I was diagnosed with an extremely rare cancer. And, it was stage 4.
My desmoplastic small round cell tumor symptoms
Looking back now, I can see some symptoms. I’d been experiencing abdominal pain for a couple of months. I have Crohn’s disease, though, so I thought it was just a flare-up, or maybe I’d pulled a muscle carrying my toddler around. But I’d also lost about 20 pounds without really trying. I felt full easily and got nauseous frequently, too. I chalked all that up to jitters about starting residency.
Unfortunately, the pain in my left side kept getting worse. It was so bad during a visit to New Mexico that I finally went to an emergency room. A CT scan revealed a large tumor in my abdomen. It was compressing the descending colon, which explained why I’d been having such trouble using the bathroom. Most of the pain I’d been experiencing was because hardly anything could pass through it.
How I got to UT MD Anderson
The ER doctors believed the growth was caused by cancer. They just weren’t sure what kind. They suspected it was either lymphoma or sarcoma and performed a biopsy to learn more. When the results came back, they told me to head straight to UT MD Anderson.
The doctors in New Mexico said the cancer cells looked so strange under a microscope that they couldn’t even identify the exact type. But it had lots of mutations, was dividing rapidly and had already outgrown its blood supply. All of those features suggested it was highly aggressive — and I needed an expert, fast.
I’m no oncologist, but I had enough medical training by then to recognize that this was very bad. The cancer was already scattered throughout my abdomen. All the medical literature I’d read indicated that once a cancer has spread in this way — regardless of what type it is — your prognosis is extremely poor.
I was stunned. My son was only 17 months old at the time. I was only 32 and still breastfeeding him. My biggest fear was that he wouldn’t remember me.
My desmoplastic small round cell tumor diagnosis
I returned home to Houston immediately. I was admitted to UT MD Anderson within a week because I arrived there in such bad shape.
I met first with Dr. Elise Nassif Haddad, a sarcoma specialist who said she thought I had sarcoma, too. I’d need some additional testing to determine exactly which kind, but my condition was serious enough that we couldn’t wait around for the details. I had to begin treatment right away.
Dr. Haddad started me on a chemotherapy regimen within days. A few weeks later, she gave me my official diagnosis. I had an exceptionally rare cancer called desmoplastic small round cell tumor (DSRCT). It’s a form of soft tissue sarcoma usually found in children. It’s so rare that fewer than 1,000 cases of it have ever even been documented. My chances of having it were about one in a billion.
My desmoplastic small round cell tumor treatment
Unfortunately, my chances of surviving this disease were not much better. Because of how many spots of cancer were already scattered around my belly, I didn’t think I was eligible for surgery. And, while I knew that chemotherapy might prolong my life, I didn’t think I would ever be cured. As it turns out, I was wrong — on both counts.
The chemotherapy regimen I’d begun turned out to be just right for treating DSRCT. So, I stayed on it and finished all six rounds Dr. Haddad recommended. Then, I met with Dr. Chris Scally, a surgical oncologist who specializes in treating sarcoma. He said I was actually a good candidate for surgery, based on my response to chemotherapy.
Dr. Scally believed he could successfully remove all the remaining cancer. I didn’t think that was even possible. But he seemed so confident that I scheduled the surgery last October. Dr. Scally got all the cancer out in just four hours.
Looking ahead with hope
My doctors at UT MD Anderson have been very clear about my prognosis. DSRCT is still a bad disease, and my chances of recurrence are quite high.
But I’ve shown no evidence of disease since my surgery last fall, which is something I never thought I’d be able to say. My doctors are both guardedly optimistic, and I’m on maintenance chemotherapy now and doing really well. I also live right next to the world’s greatest cancer center, and I get checkups every month.
Medical literature indicates that only a handful of people have lived longer than five years after receiving this diagnosis. Most people with this disease see a recurrence within 9 to 14 months of attaining remission, and I’m already at month 5. So, I don’t know if it’s realistic to hope that I can watch my son start the first grade or not.
Still, I want to see how far I can go. Things have already gone as well as they possibly could. And, thanks to UT MD Anderson, I’m already beating the odds. I am incredibly grateful.
Request an appointment at UT MD Anderson online or call 1-877-632-6789.
Why choose UT MD Anderson for your childhood soft tissue sarcoma treatment?
At UT MD Anderson's Children's Cancer Hospital, we know your child's health and well being are your number one concern. Our renowned experts customize your child's care for soft tissue sarcoma, utilizing the most advanced treatments and techniques with the least impact on your child's growing body.
Because soft tissue sarcomas are rare, most physicians have little or no experience in diagnosing and treating them. As part of one of the world's most active cancer centers, Children's Cancer Hospital has remarkable experience and skill in these types of cancer. This can make a difference in your child's outcome.
Customized care
A team of specially trained physicians follows your child throughout treatment, all the way to survivorship. They communicate closely with each other, and with you, to ensure comprehensive, personalized care. They are supported by full complement of health care professionals dedicated to your child's treatment, including nurses, physician assistants, therapists and others.
Children's Cancer Hospital offers clinical trials for innovative new treatments for soft tissue sarcoma. Behind the scenes we are working on groundbreaking basic science research to change the future of pediatric cancer.
Treating the whole child
Children's Cancer Hospital is designed just for children, with a full range of services and amenities that help make the child and family's experience as comfortable as possible. We go beyond medical care to deliver a comprehensive experience that treats the whole child.
And at Children's Cancer Hospital, your child benefits from the resources and expertise of one of the nation's top cancer centers.
Loving yourself allows you to enjoy the rollercoaster of life you’ve been given.
UT MD Anderson's Children's Cancer Hospital