Soft tissue sarcomas (STS) represent a diverse group of cancers that begin in the supportive and connective tissues of the body. These tissues surround, connect, or support the body's structure and organs.
This group of cancers includes those arising from:
Fat (adipose tissue)
Cartilage
Connective tissue (fascia, ligaments, tendons)
Muscle
Skin
Nerves
Joints
Blood vessels
Lymph vessels
The most common sites for soft tissue sarcomas are the arms and legs, but they can also develop in the head and neck, chest, abdomen, and pelvis. While soft tissue sarcomas affect both adults and children, the following information is focused on the condition as it presents in children.
Rhabdomyosarcoma is the most common form of soft tissue sarcoma found in children. It develops in the muscles that are attached to the skeleton. Each year, approximately 340 children across the nation are diagnosed with rhabdomyosarcoma. This cancer accounts for about 3% of all childhood cancers and 2% of adolescent cancers.
Other childhood soft tissue tumours are classified as non-rhabdomyosarcoma soft tissue sarcomas (NRSTS). Collectively, this group makes up approximately 4% of paediatric cancers. Many types exist and are classified according to their tissue of origin. The most common soft tissue sarcomas in children, other than rhabdomyosarcoma, include:
In children, this is typically a low-grade, slow-growing tumour. The most common type is myxoid liposarcoma.
This type develops in the cartilage outside of the bone structure, although it is more common in adults.
This is associated with the Epstein-Barr virus in children with HIV/AIDS. Survivors of retinoblastoma also have a higher risk.
These grow in the protective covering of the nerves outside of the brain and spinal cord. Children with the genetic condition neurofibromatosis type 1 (NF1) are at an increased risk.
These tumours are typically found in the joints of the legs or arms, or in the head, neck, or trunk. Children under 10 with small tumours have the best chance for successful treatment.
A risk factor is anything that increases a child's likelihood of developing soft tissue sarcoma. These factors include:
Teenagers and infants under 1 year of age are at a higher risk.
Previous radiation therapy.
It is important to remember that the presence of a risk factor does not mean your child will definitely develop the disease. If any of the above conditions are part of your family's medical history, we encourage you to inform your child's doctor, as genetic testing may be suggested.
The signs of soft tissue sarcoma can differ greatly depending on the child and the specific disease type. The location of the sarcoma also plays a significant role in the symptoms that appear.
The first sign of a soft tissue sarcoma is usually a painless mass or lump. This may be located on an arm, leg, or the trunk of the body.
As the sarcoma grows, it may press on adjacent body parts, such as nerves, muscles, blood vessels, and organs. When this occurs, other symptoms may present, including muscle weakness or pain.
Soft tissue sarcomas are rare. If your child exhibits these symptoms, they are most likely caused by a less serious condition. Nevertheless, it is always advisable to consult with a doctor to rule out other health concerns.
Soft tissue sarcomas are complex and can originate in many different parts of the body. This complexity often makes them challenging to diagnose accurately. However, an accurate diagnosis is absolutely critical to successful treatment, as an incorrect diagnosis can be detrimental.
We strongly advise that the initial biopsy, if possible, be carried out at the cancer centre where your child will be treated. It is best to choose a cancer centre that treats a large number of paediatric sarcoma patients and has a specialised team, including dedicated pathologists.
At SSCHRC, we treat more children with soft tissue sarcoma than most hospitals. Our specialist experts possess a remarkable level of skill, which helps them accurately pinpoint the tumour's location and extent. We utilise the latest diagnostic methods and technology to ensure your child receives the most accurate diagnosis possible, which can significantly influence treatment success.
If your child presents with symptoms that may suggest a soft tissue sarcoma, the doctor will examine them closely and gather details about their health and medical history, as well as the family's medical history. One or more of the following diagnostic tests may be used to confirm if your child has a soft tissue sarcoma, determine if it has spread, and check if treatment is effective:
A biopsy is the only way to be certain of a soft tissue sarcoma diagnosis. This involves removing a small sample of cells from the tumour to be examined under a microscope. Surrounding tissue and lymph nodes may also be biopsied. Imaging tests may be used before or after the biopsy to determine the exact location and extent of the tumour.
The doctor will select one of the following types of biopsy based on the tumour's location:
Several imaging tests can be used to help diagnose soft tissue sarcoma, including:
If your child is diagnosed with a soft tissue sarcoma, the medical team will discuss the best treatment strategies. The plan is always customised and carefully planned, depending on several factors:
Treatment for soft tissue sarcoma may include one or more of the following therapies.
Surgery is the primary treatment for soft tissue sarcoma. If the entire tumour can be successfully removed, the chance of a positive outcome is higher.
Sarcoma surgery, like all complex procedures, is most successful when performed by a specialist with considerable experience in that particular field. Surgeons at SSCHRC are among the most skilled and recognised globally. They are specialised in soft tissue sarcoma treatment and employ the least-invasive and most advanced surgical techniques.
During the operation, the surgeon removes as much of the tumour as possible, along with a margin of surrounding tissue to ensure the maximum removal of cancer cells. Lymph nodes may also be removed.
Your child may receive chemotherapy or radiation therapy either before or after the surgical procedure. A special type of surgery, called limb-sparing surgery, which is often followed by radiation therapy, means that the majority of patients do not require the removal of an arm or leg to treat the sarcoma.
Chemotherapy may be administered before surgery to shrink the tumour, making it easier to remove surgically. For certain types of soft tissue sarcoma, or if the cancer has spread, chemotherapy may also be given after surgery. SSCHRC offers a range of current and advanced chemotherapy options.
Advanced radiation therapy techniques and the renowned skill of SSCHRC doctors allow them to target tumours with high precision, delivering the maximum necessary amount of radiation while minimising the impact on healthy cells.
Although radiation therapy is generally not the main treatment for soft tissue sarcoma, it may be used:
SSCHRC provides the most advanced radiation treatments, including:
In some instances, the most appropriate approach may be to closely monitor your child for a period of time before initiating treatment.
SSCHRC is actively leading the future of soft tissue sarcoma treatment by developing innovative targeted therapies. These specialised agents are designed to treat a cancer's specific genetic and molecular profile, helping the body to fight the disease.
Many doctors at SSCHRC are dedicated researchers who have pioneered and actively lead national and international clinical trials involving novel targeted agents.
At SSCHRC, one of the world's foremost teams of experts customises a treatment plan for your child. This multidisciplinary group is among the few in the nation with specialised experience in treating these rare types of cancers.
SSCHRC is committed to providing the most advanced treatments for soft tissue sarcoma with the least impact on your child's body, both today and in the future.
The specialised team that treats your child includes highly trained doctors such as oncologists, surgical oncologists, and radiation oncologists. They are supported by a group of dedicated professionals, including advanced practice nurses, physician assistants, therapists, and social workers.
Since surgery is often the main treatment, and the procedures can be delicate and complex, our surgeons are among the most skilled in the world in soft tissue sarcoma surgery. They employ the latest techniques and technology to give your child the best possible chance for successful treatment.
SSCHRC offers a range of clinical trials for soft tissue sarcomas, keeping us at the forefront of discovering new and better treatment methods, which translates to better chances for successful treatment for your child.