Targeted cancer drugs for soft tissue sarcoma

Targeted cancer drugs work by ‘targeting’ those differences that help cancer cells survive and grow. You might have them as part of your treatment for soft tissue sarcoma. 

What are targeted cancer drugs?

Cancer cells have changes in their genes (DNA) that make them different from normal cells. These changes mean that they behave differently. Cancer cells can grow faster than normal cells and sometimes spread.

The changes in genes that cause one cancer type are often different to the genetic changes causing another. For example, the changes that make a sarcoma grow can be different to ones that make breast cancer grow.

Targeted drugs target those differences that help a cancer to grow. There are many different targets on cancer cells and different drugs that target them. 

The different types work in one (or sometimes more than one) of the following ways:

  • stop cancer cells from dividing and growing
  • seek out cancer cells and kill them
  • encourage the immune system to attack cancer cells
  • stop cancers from growing blood vessels
  • help carry other treatments such as chemotherapy, directly to the cancer cells

You might hear some targeted drugs called biological therapies.

When you might have targeted cancer drugs for soft tissue sarcoma

Targeted cancer drugs are used to try to shrink or control soft tissue sarcoma. This aims to help you live longer. These drugs often stop or slow the growth of your cancer for months, and sometimes years.

Different treatments work for different patients. Your doctor will look at your general health to decide on the best treatment for you. 

Types of targeted cancer drugs for soft tissue sarcoma

Imatinib (Glivec)

Imatinib is used to treat a type of soft tissue sarcoma called a gastrointestinal stromal tumour (GIST). It is a type of targeted drug called a tyrosine kinase inhibitor (TKI).

Tyrosine kinase inhibitors (TKIs) block chemical messengers (enzymes) called tyrosine kinases. Tyrosine kinases help to send growth signals in cells. So, blocking them stops the cell from growing and dividing.

You might have imatinib to treat a GIST:

  • that can’t be completely removed with surgery
  • which has spread before surgery

Some people with GIST have a higher risk of their cancer coming back after surgery. This is called a high risk GIST. Imatinib can help to reduce the chances of the GIST coming back. So in this situation, your doctor may recommend you take imatinib for up to 3 years after your operation.

You may also have imatinib to shrink a GIST before surgery so that your surgeon can remove it more easily. Sometimes a surgeon can completely remove a GIST after treatment with imatinib. 

Imatinib may also be used to treat a very rare, low grade type of sarcoma called a dermatofibrosarcoma protruberans (DFSP). DFSP develops in the skin.

Sunitinib (Sutent)

Sunitinib is also a tyrosine kinase inhibitor (TKI). Your doctor might recommend that you have sunitinib (Sutent) in one of the following situations:

  • imatinib has stopped working
  • you have had severe side effects with imatinib treatment

You must have GIST that cannot be completely removed or has spread.

Regorafenib (Stivarga)

Regorafenib (Stivarga) is used to treat advanced GIST. Like the above drugs, it is a tyrosine kinase inhibitor (TKI). Your doctor might recommend regorafenib if you have had treatment with imatinib and sunitinib and these drugs:

  • have not worked
  • or caused bad side effects

Advanced GIST means you cannot have surgery to remove the GIST, or it has spread. You must be fairly fit and well to have this drug.

Larotrectinib (Vitrakvi)

Larotrectinib is used to treat some types of sarcoma which test positive for the neurotrophic tyrosine receptor kinase (NTRK) fusion gene. The NTRK fusion gene can also be in many other types of solid tumours Open a glossary item.

A fusion gene Open a glossary item means a change (mutation) that happens when a piece of the chromosome Open a glossary item that carries a gene called NTRK breaks off. It then joins (fuse) with a gene on another chromosome. NTRK gene fusions lead to abnormal proteins called TRK fusion proteins. These proteins may cause cancer cells to grow.

You might have larotrectinib if:

  • your cancer has spread to nearby tissues or other parts of the body
  • surgery could cause severe health problems
  • there are no other treatment options

You have larotrectinib as part of the Cancer Drugs Fund.

Entrectinib (Rozlytrek)

Entrectinib is another drug used to treat some types of sarcoma which tests positive for the neurotrophic tyrosine receptor kinase (NTRK) fusion gene.

You might have entrectinib if:

  • your cancer has spread to nearby tissues or other parts of the body
  • surgery could cause severe health problems
  • you have not had an NTRK inhibitor before
  • there are no other treatment options

You have entrectinib as part of the Cancer Drugs Fund.

Having targeted cancer drug treatment for soft tissue sarcoma

You have your treatment as tablets or capsules.

You must take tablets and capsules according to the instructions your doctor or pharmacist gives you.

You should take the right dose, not more or less.

Talk to your specialist or advice line before you stop taking a cancer drug.

Side effects

The side effects you have depend on:

  • which drug you have
  • whether you have it alone or with other drugs
  • the amount of drug you have (the dose)
  • your general health

Everyone is different and the side effects vary from person to person. You might have a few side effects. And they may be mild or more severe. 

A side effect may get better or worse during your course of treatment. Or more side effects may develop as the treatment goes on. 

For more information about the side effects of your treatment, go to the individual drug pages.

When you go home

Treatment with targeted drugs can be difficult to cope with for some people. Your nurse will give you a number to call (advice line) if you have any problems at home.

Contact your advice line if you have side effects or any concerns.
Last reviewed: 
27 Jul 2021
Next review due: 
29 Jul 2024
  • UK guidelines for the management of soft tissue sarcomas
    A Dangoor and others
    Clinical Sarcoma Research, 2016. Volume 6, number 20 

  • UK clinical practice guidelines for the management of gastrointestinal stromal tumours (GIST)
    I Judson and others
    Clinical Sarcoma Research, 2017. Volume 7, Issue 6, pages 1 to 10

  • A systematic review and meta-analysis of neurotrophic tyrosine receptor kinase gene fusion frequencies in solid tumors

    A Forsythe and others

    Therapeutic Advances in Medical Oncology, 2020, volume 12: 1-10

  • Various guidelines from the National Institute for Health and Care Excellence and Scottish Medicines Consortium website
    Accessed July, 2021

  • Current Molecular Targeted Therapies for Bone and Soft Tissue Sarcomas

    K Nakano and others 

    International journal of meloecular sciences (2018), volume 19, number 739

  • Novel Insights into the Treatment of Imatinib-Resistant Gastrointestinal Stromal Tumors

    C Serrano and others 

    Targeted oncology (2017), volume 12, number 3, pages 277-288

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