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Malignant schwannoma

Malignant schwannomas are also called malignant peripheral nerve sheath tumours (MPNSTs) or neurofibrosarcomas.

Most schwannomas are not cancerous (a benign tumour). A tumour is a lump or growth in the body. This information is about schwannomas that are cancerous (malignant).

What is a malignant schwannoma?

Malignant schwannomas are a rare type of cancerous tumour that are classed as a soft tissue sarcoma. A sarcoma is a type of cancer. Soft tissue is a term used to describe all the supporting tissues in the body, apart from the bones. So this includes muscle, nerves, tendons and deep skin tissue. 

In over half of all cases they are linked to a condition called Neurofibromatosis type 1. Most of the other cases of malignant schwannoma occur by chance (randomly).

Tests to diagnose malignant schwannoma

Your specialist will examine you and then you may need other tests which can include:

  • blood tests
  • ultrasound scan
  • MRI scan
  • CT scan
  • X-ray

Treatment of malignant schwannoma

Malignant schwannoma can be difficult to treat.


The main treatment is surgery. Your surgeon aims to remove as much of the cancer as possible and the surrounding tissue. 


You might have radiotherapy before surgery. Radiotherapy aims to shrink the cancer, making it easier to remove. This is called neoadjuvant treatment. 

Radiotherapy after surgery can kill any cancer cells left behind, if your surgeon wasn't able to remove all the cancer. It also aims to lower the risk of sarcoma coming back. This is callled adjuvant treatment. 


Malignant schwannomas don't respond very well to chemotherapy. Chemotherapy may be used to try to shrink the tumour or slow it's growth, but it is usually unlikely to cure it. 

Malignant schwannoma that comes back

Malignant schwannoma that comes back in the same place is called local recurrence.

Your surgeon aims to remove all the cancer during surgery. Sometimes this isn't possible. It depends on the size and position of the sarcoma. Occasionally there are microscopic traces of cancer left behind. This is enough for it to grow into a new tumour.

It can be more difficult to treat if the sarcoma comes back. You might have surgery again if it possible to remove the new tumour. You might also have radiotherapy again depending on the previous radiotherapy dose.


Being diagnosed wtih cancer is hard to cope with. A lot of people with rare cancers also say they feel isolated.

It can be more difficult to find information about rare cancers and to find other people who have the same type of cancer as you.

Talking to other people going through something similar can be helpful, even if they don’t have exactly the same cancer type.


Coping with a diagnosis of a rare  cancer can be especially difficult, both practically and emotionally. Being well informed about your sarcoma and its treatment can make it easier to make decisions and cope with what happens.

Sarcoma UK provides support and information for people affected by soft tissue and bone sarcoma.

The Rare Cancer Alliance offer support and information to people affected by rare cancers.

Talking to other people who have the same thing can also help.

Our discussion forum Cancer Chat is a place for anyone affected by cancer. You can share experiences, stories and information with other people who know what you are going through.

Last reviewed: 
09 Jul 2018
  • Malignant peripheral nerve sheath tumors state of the science: Leveraging clinical and biological insights into effective therapies

    A R Kim and others.

    Sarcoma. 2017;2017:7429697

  • Update from the 2011 International Schwannomatosis Workshop: From genetics to diagnostic criteria

    SR Plotkin and others

    American Journal of Medical Genetics, 2013. Volume 161, Issue 3

  • Neurofibromatosis: A review of NF1, NF2, and Schwannomatosis

    JL Kresak and M Walsh

    Journal of Pediatric Genetics, 2016. Jun;5, (2) , pages 98-104

  • UK guidelines for the management of soft tissue sarcomas

    A Dangoor and others

    Clinical Sarcoma Research, 2016. volume 6, number 20

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