​Soft tissue tumours arise from soft tissues such as muscles, nerves, blood vessels, and fat and may involve any area of the body. While not all soft tissue tumours are malignant (cancerous), when the term 'sarcoma' is used, it implies that the tumour is malignant.

People of any age can be affected with sarcoma, although it tends to occur in adults more often than children. A painless lump is the most common symptom, especially when found in the early stages.

At times, the sarcoma may be found after attention is drawn to the area from an unrelated injury. Symptoms such as pain, numbness, swelling, or difficulty breathing can arise if the sarcoma compresses adjacent tissues and organs. Constitutional symptoms such as fever or weight loss can also be present.

Imaging studies help define the local and systemic (rest of the body) extent of the disease and include plain radiographs (x-rays), MRI, and CT scans. A small tissue sample (biopsy) allows for confirmation of the diagnosis. Surgery, radiotherapy, chemotherapy, and more recently, targeted agents, either alone or in combination, are typical treatment options.

Notably, there are many different subtypes of sarcoma that can behave and respond differently to treatment. The complexity of therapy dictates that patients with sarcomas should seek treatment in a tertiary centre with comprehensive multidisciplinary care.

In July's ‘Ask the Specialist’ forum, Dr Looi Wen Shen, Consultant from the Division of Radiation Oncology at National Cancer Centre Singapore (NCCS), a member of the SingHealth group, answers your questions on sarcomas.

This 'Ask the Specialist' forum has closed. Thank you for your interest and participation.

 

1. Question by JC
Hi Dr Looi,
Here are my questions on sarcoma:

1.How common are sarcomas in Singapore? Is it more common in men or women?
2.What are common causes and what factors put me at risk?
3.Are there different stages and what are the survival rates?
4.What can I do to reduce my risk?

Thank you for your advice.

Answered by Dr Looi:
Dear JC,
Sarcomas are rare worldwide and account for approximately 1% of all adult solid malignant tumours. There isn’t a strong predisposition for either gender.

Most of the time, a clear reason for why a soft tissue tumour develops is not known. However, patients with certain medical conditions may be more likely to develop soft tissue sarcomas.

These conditions include neurofibromatosis, Li-Fraumeni syndrome, Gardner's syndrome, Werner's syndrome, nevoid basal cell carcinoma syndrome, and retinoblastoma. Additionally, exposure to radioactivity and certain chemicals such as arsenic and alkylating agents are known to increase risk.

Broadly speaking, soft tissue sarcomas can be divided into those that are localised, locally advanced, and metastatic. As many different types of soft tissue sarcoma exist, I cannot provide definite survival statistics.

Most sarcomas develop in people with no known risk factors and there is little that can be done in terms of lifestyle choices. No screening tests or exams are recommended for the general population. However, if you have a strong family history of sarcomas and/or known genetic syndromes, you can consider speaking to a doctor about genetic testing.


2. Question by alex_h
Hi Dr Looi,
My understanding is that there are many different types of sarcomas, is that right? As a layman, are there major types that I need to be aware of which are the most common? Thanks.

Answered by Dr Looi:
Dear Alex_h,
There are more than 80 types soft tissue sarcoma or sarcoma-like growths; a biopsy is required to determine what subtype it belongs to. For a layperson, it is important to know when to seek medical attention. If you have an enlarging lump (especially 5cm or larger), it would be good to see a doctor. This is especially so if the lump feels deep-seated, starts to become painful, or has recurred after a previous surgery.


3. Question by csang70
Dear Dr Looi,
Can sarcoma spread quickly to other parts of the body? What are the likely areas that sarcoma spreads to? Can it be cured completely? As for soft tissue sarcoma, is it possible to die from it? Thanks for your advice.

Answered by Dr Looi:
Dear csang70,
If the tumour does spread to other parts of the body, the most commonly affected is the lungs. However, other sites such as lymph nodes, soft tissues, bone, liver, brain, and even skin are possible; the sites of involvement are influenced by the type of sarcoma. Localised tumours and sometimes even locally advanced tumours are potentially curable. 

Surgery is generally the main curative therapy if the disease can be removed completely; radiotherapy and chemotherapy or other newer systemic therapies may be recommended as additional therapies.

Although metastatic disease may not always be curable, there are treatment options available. As with all cancers, unfortunately, it is possible for patients to succumb to the disease.


4. Question by Suzy
Dear Dr Looi Wen Shen,
What are the treatment options for sarcoma? Is surgery usually the first course of action? What can I do after to minimise the risk of recurrence? Thank you.

Answered by Dr Looi:
Dear Suzy,
Surgery is generally the main curative therapy if the disease can be removed completely; radiotherapy and chemotherapy or other newer systemic therapies may be recommended as additional therapies.

Although metastatic disease may not always be curable, there are still treatment options available. There are no lifestyle changes you can make to reduce the risk of recurrence. However, if you have been treated for sarcoma, you can speak to your doctor about the symptoms you might experience if a recurrence occurs so that you can monitor for these.


5. Question by Wai Cheng
Hi Dr Looi, here are my questions:
I would like to know more about rhabdomyosarcoma.
1.Is it potentially curable? 
2.What are the treatment modalities for such cases?
3.What is the prognosis for advanced stage rhabdomyosarcoma?
It would be greatly appreciated if you could answer them. Thank you for your advice.

Answered by Dr Looi:
Dear Wai Cheng,
Thank you for your questions.

Regarding 1) and 3) 
◦The clinical outcome, including the chance of cure, is dependent on multiple factors, including the location, subtype, and stage of the disease at presentation. 

Regarding 2) 
◦This depends on the location, whether the tumour can be surgically removed, subtype, and stage. Based on these variables, treatment may involve chemotherapy, surgery, and possibly radiotherapy.


6. Question by Lee
Dear Dr Looi,
My family member has a long existing lump on the leg which gives no pain or changes in shape, colour or size. May I know if this is a risk for sarcoma and any action required? Thank you.

Answered by Dr Looi:
Dear Lee,
Thank you for your question. Although the lump has remained stable, it would be helpful for your family member to see a physician who can perform a clinical examination that will provide more clues about the diagnosis of the condition.


7. Question by dornkel
Dear Dr Looi, 
I have 2 lumps at the back of my neck right below the skin close to my spine with the size of about 3-5mm. I also have numerous lumps about the same size over my body. How do I know if they are malignant? The 2 behind my neck sometimes irritates me. Can I have them removed?

Thanks for your advice.

Answered by Dr Looi:
Dear dornkel,
Thank you for your question. It would be useful for you to see your primary care provider, who can perform a clinical examination and determine if further investigations are warranted.


8. Question by Helen L
Dear Dr Looi,
What is localised, locally advanced and metastatic?

If one has completed hysterectomy and subsequent removed some nodules in lungs, done with chemotherapy as well as oral targeted drugs and has been cleaned for about a year now - when will the patient be declared cancer-free? May I know have you come across any patients survived and lived long?　
Any advice to keep it (diet, exercise?) at bay for good?
Thank you for your kind advice.

Answered by Dr Looi:
Dear Helen L,
Thank you for your questions.
1.Generally speaking, localised means that the sarcoma is in only one area of the body; locally advanced means that the sarcoma involves nearby tissues or organs; metastatic means that the sarcoma has spread to parts of the body that are distant from the original site of the sarcoma.
2.In patients with metastatic disease, treatment usually does not cure, except in certain situations. Most often, the aim of treatment is to slow the growth of disease and reduce symptoms. It is important for patients to have a discussion with their physicians about their disease status and the goals of treatment.
3.Extended survival in patients with metastatic disease is not unheard of.
4.Although not specific to sarcoma, there is a growing body of evidence that regular exercise may improve survival and quality of life in cancer patients.


About Dr Looi Wen Shen

Dr Looi Wen Shen is a Consultant radiation oncologist in the Department of Radiation Oncology at the National Cancer Centre Singapore (NCCS), and a Clinical Assistant Professor in Duke-NUS medical school. He is fellowship-trained, having completed a year-long fellowship in paediatric and adult proton therapy at the University of Florida Health Proton Therapy Institute. He specialises in caring for both adults and children, and has an interest in paediatric tumours, urologic tumours, sarcomas, and lymphomas.

After receiving his undergraduate medical degree from Yong Loo Lin School of Medicine, National University of Singapore, Dr Looi went on to complete his residency training in National Cancer Centre Singapore before receiving specialist accreditation in radiation oncology by the Specialist Accreditation Board. He is an active cancer researcher and his research has been published in respected journals such as the International Journal of Radiation Oncology Biology and Physics, and Medical Physics, Paediatric Blood and Cancer, and the Archives of Disease in Childhood. Through his research, he endeavours to decrease the acute and late side effects of radiation in children and adults, and improve their quality of life.

Because #healthiswealth #healthforgood

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