Sarcoma

• Gastrointestinal stromal tumours (GISTs) – usually forms in the digestive tract, tend to be large and can spread to other parts of the body
• Liposarcoma – originates in fatty tissue with some types being more aggressive than others
• Leiomyosarcoma – forms in the smooth muscle tissue that lines hollow organs. In women, the uterus is the most common location for this type of sarcoma.
• Undifferentiated pleomorphic sarcoma (UPS) – can form anywhere, usually occurs in the abdomen, on arms and legs (especially thighs), and grows and spreads quickly to other parts of the body.
• Synovial sarcoma – more common in children and young adults. Usually occurs on the arms, legs, finger and toes.

• Osteosarcoma – also called osteogenic sarcoma, these begin in cells within the bones and often occur in the arms and legs
• Chondrosarcoma – starts in the cartilage, often found in the pelvis, upper leg and shoulder bones
• Ewing sarcoma – can occur in any bone and can also occur in soft tissue, most commonly affects the pelvis, thigh, lower leg, upper arm and chest wall
• Chordoma – a rare form of bone sarcoma that can occur in the skull or spine
• Giant cell tumours – a rare form of bone sarcoma that can grow quickly and become cancerous, often forms near the joints

In the early stages, patients with sarcomas may not have any noticeable symptoms. In later stages, symptoms may include:

• Weight loss
• Loss of appetite
• Prolonged fever
• Long-lasting bone pain
• Noticeable lumps, masses or growths • Swelling in the arms or legs that get worse at night • Symptoms related to the site of sarcoma such as fullness upon eating, indigestion, and gastric pain when a stomach sarcoma is present, or bleeding from the vagina when a uterus sarcoma is present

When to see a doctor:

• Noticeable lumps, masses or growths
• Painless masses that become painful
• Masses or growths that increase in size
• Masses larger than a golf ball (around 5cm)
• If a child has bone pain that does not get better and that did not occur as a result of injury

There are very few known risk factors for sarcomas and patients do not always present with them. However, some causes and risk factors may include:

• Family history of cancer-related syndromes such as neurofibromatosis. Neurofibromatosis can cause multiple benign enlargements of nerve sheaths, leading to a higher risk of one of these enlarged nerve sheaths turning cancerous. Other syndromes that may increase risk are Li-Fraumeni syndrome, familial adenomatous polyposis, Carney-Stratakis syndrome, hereditary retinoblastoma, neurofibromatosis and BRCA2 gene mutations.
• Past radiotherapy treatment can lead to a slightly higher chance of developing a sarcoma within the previously radiated area. This can occur years later.
• Exposure to certain toxic chemicals such as industrial chemicals or herbicides.
• A damaged lymphatic system, the network of tissues, vessels and organs that are an important part of the immune system.

Sarcoma patients usually consult a doctor after noticing a lump or mass on the abdomen, trunk, arms of legs. The lump may or may not be painful. There are several tests that can be used to diagnose sarcoma and determine whether it has spread.

Tests and procedures used to diagnose sarcoma:

• Biopsy
• A sample is taken by removing a piece of tissue from the lump/mass for examination and testing in the laboratory.
• MRI/CT scans
• Once a sarcoma is diagnosed, imaging tests are usually ordered to determine the spread and guide treatment. - Magnetic resonance scanning (MRI) of the site of the sarcoma can show the extent of the cancer and if it is operable - Computerised scans (CT) of the lungs and liver can determine if the cancer has spread.

An individual with sarcoma should be assessed by a multi-disciplinary team of specialists to determine which modalities of treatment are best suited for them. All sarcoma cases seen at SingHealth institutions are reviewed at a weekly multidisciplinary tumour board, where decisions on treatment are made following discussions, so that care is holistically managed.

Surgery

Patients who have sarcoma localised to the organ of origin would undergo surgery to remove the entire cancer and surrounding normal tissue, where possible. In some patients, additional radiotherapy to the site of cancer is required after surgery, to prevent cancer recurrence at the same site. This can happen if the cancer mass is large to begin with. In some particular subtypes of sarcoma, additional chemotherapy may also be required after curative surgery.

Radiotherapy

Radiation or radiotherapy uses high-energy X-rays to kill cancer cells. The aim of radiotherapy is to destroy cancer cells and spare as many normal cells as possible. For sarcoma patients, radiotherapy to the arms and legs is associated with redness of the skin and hair loss over the radiated area. Radiotherapy to the head and neck region may cause sides effects such as temporary redness of the skin, loss of appetite, loss of salivation and dryness of the mouth. Radiotherapy to the stomach region may cause temporary nausea and vomiting, loss of appetite, and occasional gastric pain. Your doctor will discuss with you how to manage these side effects.

Chemotherapy

Chemotherapy is the use of anti-cancer drugs to destroy cancer cells. Patients with osteogenic sarcoma usually require chemotherapy to prevent cancer recurrence after surgery. This chemotherapy is administered into the veins in the hand and may cause nausea and vomiting, temporary hair loss, lowered blood count and occasional numbness of the fingers and toes. Some patients with localised soft tissue sarcoma of the limbs benefit from chemotherapy administered post-surgery.

In patients with advanced stage sarcoma, chemotherapy can slow the course of the cancer and provide relief from cancer-related symptoms. Side effects may include temporary or complete hair loss, nausea and vomiting, ringing of the ears, numbness of the fingers and toes, and loss of appetite. More serious side effects may include organ dysfunction (including the heart, kidney and gastrointestinal tract) and serious infections as a result of bone marrow suppression. Your doctor will discuss with you how to manage these side effects.

Adjuvant therapy

One particular subtype of sarcoma known as gastrointestinal stromal tumour (GIST), which usually starts in the digestive tract, can be treated with an oral medication known as Imatinib. This relatively new medication specifically targets the mutated protein that causes the cancer, as opposed to chemotherapy that affects both normal and cancerous cells. Imatinib is used both after curative surgical removal of localised GIST, and in advanced GIST.

Immunotherapy

Immunotherapy is a drug treatment that trains your immune system to fight the cancer.

Before surgery, your surgeon will perform comprehensive medical assessments including blood tests and scans to see if you are suitable for surgery and advise on the risks involved. Your treatment recommendation is often based on consensus by a multi-disciplinary team known as the tumour board, who come together to discuss the pros and cons of every treatment strategy recommended.

Before surgery, the anaesthesia team will assess your fitness for surgery and advise you on various aspects of general anaesthesia and pain control after surgery.

Specialist nurses will provide pre-surgery counselling so that you know what to expect.

After surgery, you will be given regular outpatient appointments to see your team of doctors. During these appointments you may have blood tests and scans to check if the cancer recurs.

It is important to follow your doctor's advice, keep to your clinic visits and do the recommended scans and tests, so that timely treatment can be administered if the cancer recurs or other problems occur.

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