Soft tissue sarcomas are a rare group of cancers that develop in the soft tissues that surround, connect, or support the body's structures and organs. These tissues include muscles, joints, tendons, fat, blood vessels, nerves, and other connective tissues.
While some soft-tissue growths, such as lipomas and haemangiomas, are benign (non-cancerous), others are malignant (cancerous) and are referred to as soft tissue sarcomas. There are over 30 recognised types of sarcoma, which contributes to their rarity. Sarcomas are classified into groups based on similar cancer cell types and symptoms, and they are typically named after the tissue where they originate. Sarcomas within the same classification are often treated similarly.
Muscle tissue
Peripheral nerve tissue
Joint tissue
Blood and lymph vessels
Fibrous tissue
The connective tissue of the breast, often in the form of phyllodes tumours and other soft tissue sarcomas
A small number of these cancers are classified as Sarcomas of uncertain tissue type when specialists are unable to determine the exact tissue of origin.
A risk factor is anything that increases the likelihood of developing cancer. For sarcoma, risk factors include:
Von Recklinghausen disease
Li-Fraumeni syndrome
Gardner syndrome
Inherited retinoblastoma
Werner syndrome
Gorlin syndrome
Tuberous sclerosis
Damage to or removal of lymph nodes during previous cancer treatments
Exposure to vinyl chloride, a chemical used in the manufacturing of plastics
Previous radiation treatment for another type of cancer
It is important to note that not everyone with these risk factors will develop sarcoma. If you have any of these risk factors, it is advisable to discuss them with your healthcare provider.
The signs and symptoms of sarcoma can vary widely from person to person. Often, sarcoma presents with no noticeable symptoms in the early stages, which is why only about half of soft-tissue sarcomas are detected before they spread.
The symptoms a patient experiences often depend on the tumour's location:
A patient may notice a lump that grows over a period of weeks to months. The lump may or may not be painful.
(the back wall inside the abdomen): Tumours here may cause symptoms such as pain, or they may grow large enough to be felt in the abdomen. They can also lead to blockages or bleeding in the stomach or bowels.
You should consult your doctor if you experience any of the following:
A new lump or a lump that is growing anywhere on your body
Abdominal pain that is progressively worsening
Blood in your stool or vomit
Black, tarry stools (which may indicate internal bleeding)
These signs do not automatically confirm a sarcoma diagnosis, but it is essential to discuss any new or worsening symptoms with your doctor as they may signal other health concerns.
An accurate diagnosis of soft tissue sarcoma is critical as it directly impacts the success of the treatment plan. Sarcomas can be challenging to diagnose due to their complexity and potential to develop in numerous locations throughout the body.
The expert team of specialised surgeons and pathologists at SSCHRC is highly experienced in diagnosing sarcomas. We are one of the few centres globally with doctors who focus exclusively on sarcoma diagnosis, ensuring the most precise and accurate results possible.
The only definitive way to confirm that a tumour is a soft tissue sarcoma is through a biopsy, which involves removing a small sample of cells or tissue for examination under a microscope. Imaging tests are often used both before and after a biopsy to determine the tumour's exact location and its extent.
The type of biopsy performed depends on the tumour's location:
Important: It is generally recommended that the surgeon who performs the biopsy is also the surgeon who will remove the tumour, as an incorrectly performed biopsy can potentially complicate treatment. Whenever possible, the initial biopsy should be performed at the cancer centre where the patient intends to receive treatment.
Imaging tests used in diagnosis and staging may include:
CT or CAT (Computed Axial Tomography) scans
MRI (Magnetic Resonance Imaging) scans
PET (Positron Emission Tomography) scans
Chest X-ray
Ultrasound
Following a diagnosis, the doctor will determine the stage of the disease, which refers to its extent. Staging is vital as it helps the doctor select the most appropriate treatment and determine the likely outlook for recovery (prognosis). Once the initial classification is determined, the stage remains the same, even if the cancer shrinks or spreads later on.
Sarcomas are often staged using the American Joint Committee on Cancer's TNM system:
For soft tissue sarcomas, an additional factor called Grade (G) is also included in the staging system. The grade is based on how the sarcoma cells look under the microscope and is a predictor of how quickly the tumour is likely to grow and spread.
The official staging system divides sarcomas into three grades (1 to 3). A pathologist determines the grade by considering three factors:
1. Differentiation
How closely the tumour cells resemble normal tissue (on a scale of 1 to 3)
2. Mitotic Count
How many cells appear to be dividing (on a scale of 1 to 3)
3. Tumour Necrosis
How much of the tumour tissue is dead or dying
These factors are scored and summed to determine the final grade:
GX: Grade cannot be assessed
Grade 1 (G1)
Total score of 2 or 3 (Low-grade: slow-growing, lower chance of spread, better prognosis)
Grade 2 (G2)
Total score of 4 or 5
Grade 3 (G3)
Total score of 6 or higher (High-grade: tends to grow and spread faster)
Low-grade tumours often have a more favourable outlook than high-grade tumours. Certain rare sarcomas, such as synovial and embryonal sarcoma, are automatically considered high-grade.
Stage grouping combines the information from the T, N, M, and G factors to assign a final stage (I to IV, often with A or B sub-stages).
Stage IA
T1, N0, M0, G1 or GX. Tumour is 5 cm or less, no spread to lymph nodes or distant sites, Grade 1 or cannot be assessed.
Stage IB
T2, N0, M0, G1 or GX. Tumour is greater than 5 cm, no spread to lymph nodes or distant sites, Grade 1 or cannot be assessed.
Stage IIA
T1, N0, M0, G2 or G3. Tumour is 5 cm or less, no spread to lymph nodes or distant sites, Grade 2 or 3.
Stage IIB
T2, N0, M0, G2. Tumour is greater than 5 cm, no spread to lymph nodes or distant sites, Grade 2.
Stage III
T2, N0, M0, G3: Tumour is greater than 5 cm, no spread to lymph nodes or distant sites, Grade 3. OR Any T, N1, M0, any G: Tumour is any size and any grade, has spread to nearby lymph nodes (N1), but not to distant sites.
Stage IV
Any G, Any T, Any N, M1. Tumour has spread to distant organs or tissues (such as the lungs) and can be any size or grade, with or without spread to local lymph nodes.
Soft tissue sarcomas are typically treated with a combination of therapies, which may include surgery, chemotherapy, and radiation therapy. Upon diagnosis, your doctor will discuss the best options, which are determined by several factors:
Treatment plans at SSCHRC are customised to the individual needs of each patient and may involve one or more of the following treatment types.
Surgery is the main treatment for soft-tissue sarcomas. The goal is to remove the tumour along with a margin of healthy tissue surrounding it to ensure as many cancer cells as possible are excised. Patients may receive chemotherapy or radiation either before or after the surgical procedure.
The success of sarcoma surgery is highest when performed by a specialist with extensive experience in the procedure. The surgeons at SSCHRC are highly skilled and utilise the least-invasive and most advanced techniques. Due to special procedures like limb-sparing surgery, often followed by radiation therapy, most patients can avoid the loss of an arm or leg.
SSCHRC offers the most advanced and up-to-date chemotherapy options. Chemotherapy may be used as the primary treatment or in combination with surgery or radiation. A treatment regimen may involve a combination of two or more chemotherapy drugs. Specialised techniques, such as limb perfusion, may sometimes be used to deliver a more concentrated dose of chemotherapy to a specific area.
New techniques and skilled doctors at SSCHRC allow for precise targeting of tumours, delivering the maximum necessary radiation dose while minimising damage to surrounding healthy cells.
Radiation therapy is typically not the main treatment but is frequently used:
SSCHRC provides advanced radiation treatments, including:
Brachytherapy
Small radioactive seeds are placed directly into the body, close to the tumour.
3D-Conformal Radiation Therapy
Multiple radiation beams are shaped to match the exact form of the tumour.
Intensity-Modulated Radiotherapy (IMRT)
Treatment is precisely tailored to the specific shape and size of the tumour.
Unlike traditional treatments that directly kill cancer cells, targeted therapy works by stopping or slowing the cancer's growth or spread at a cellular level. Cancer cells depend on specific molecules (often proteins) to survive, multiply, and spread. Targeted therapies are designed to interfere with or 'target' these molecules or the cancer-causing genes that produce them. Your doctor may request specific genomic or molecular profiling to help identify the best targeted therapies for your disease.
SSCHRC is dedicated to providing patients with the most advanced sarcoma treatments available while minimising the impact on the body. Sarcoma treatment at our centre is highly customised and delivered by a world-class team of experts.
Sarcoma is a rare disease, meaning many oncologists may treat only a few cases in their careers. Statistics indicate that sarcoma patients have better outcomes when treated at large comprehensive cancer centres, where specialised oncologists have greater experience with the disease. SSCHRC treats more sarcoma patients than many other cancer centres, resulting in an exceptional level of experience and expertise.
Our surgeons are highly skilled and use the latest techniques, including limb-sparing surgeries, which help patients avoid the loss of a limb. SSCHRC has been instrumental in clinical advancements, including the pivotal clinical trial for Gleevec® (imatinib) for GIST (gastrointestinal stromal tumours), discovering the activity of Gemzar® (gemcitabine) in soft-tissue sarcomas, and developing concurrent chemo and radiation regimens to improve the chances of successful treatment in certain types of sarcoma.