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Malignant Nerve Sheath Tumors

What is Malignant Nerve Sheath Tumors?

Malignant peripheral nerve sheath tumor (MPNST) is a rare cancer type that affects the protective tissue that surrounds the nerves that come out the spinal cord into the different parts of the body (peripheral nerves). These are the nerves that are outside the central nervous system. The presence of the tumor can cause a lump in the skin, pain in the area and weakness of movement in the affected part. These tumors can spread (metastasize) to other areas of the body.

They are considered aggressive tumors because there is a high proportion of recurrence, the tumor is able to come back after it has been removed, and it is able to extend and invade other parts of the body (usually the lungs).

 

 

Synonyms

  • Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumor (MPNST) is a rare cancer type that affects the protective tissue that surrounds the nerves that come out the spinal cord into the different parts of the body (peripheral nerves). These are the nerves that are outside the central nervous system. The presence of the tumor can cause a lump in the skin, pain in the area and weakness of movement in the affected part. These tumors can spread (metastasize) to other areas of the body.

They are considered aggressive tumors because there is a high proportion of recurrence, the tumor is able to come back after it has been removed, and it is able to extend and invade other parts of the body (usually the lungs).

 

Acknowledgement of Malignant Nerve Sheath Tumors has not been added yet.

MPNST is sometimes found in association with another disorder called neurofibromatosis type 1.  The frequency of MPNST alone in the general population is about one per 1100,000 individuals.

Name Abbreviation
Malignant Peripheral Nerve Sheath Tumors MPNST

The underlying cause is the appearance of mutations in the cells that form the tissue lining the nerves. These mutations make them grow uncontrollably, but no particular type of genetic alteration has been linked to MPNST. However, there are some risk factors associated with the development of MPNST:

  • The application of radiation therapy to treat another cancer might cause the development of MPNST in the area decades after its administration

  • The presence of benign nerve tumors

  • Individuals that suffer from neurofibromatosis type I are more prone to develop MPNSTs

Symptoms for Malignant Nerve Sheath Tumors has not been added yet.
Diagnosis of Malignant Nerve Sheath Tumors has not been added yet.
Diagnostic tests of Malignant Nerve Sheath Tumors has not been added yet

As these tumors can spread (metastasize) to other areas of the body, they may be removed surgically.  Radiation therapy is used to decrease the chance of recurrence. Chemotherapy may be used if the tumor cannot be completely removed by surgery.

Prognosis of Malignant Nerve Sheath Tumors has not been added yet.
Tips or Suggestions of Malignant Nerve Sheath Tumors has not been added yet.
References of Malignant Nerve Sheath Tumors has not been added yet.
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After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

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