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Malignant Nerve Sheath Tumors

What is Malignant Nerve Sheath Tumors?

Malignant peripheral nerve sheath tumor 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.

these tumors may be removed surgically. Sometimes, radiation therapy is necessary to decrease the chance of recurrence. Chemotherapy may be used if the tumor cannot be completely removed by surgery.

They are considered aggresive 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).

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

 

Synonyms

  • Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumor 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.

these tumors may be removed surgically. Sometimes, radiation therapy is necessary to decrease the chance of recurrence. Chemotherapy may be used if the tumor cannot be completely removed by surgery.

They are considered aggresive 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).

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

Acknowledgement of Malignant Nerve Sheath Tumors has not been added yet.
Prevalence Information of Malignant Nerve Sheath Tumors has not been added yet.
Name Abbreviation
Malignant Peripheral Nerve Sheath Tumors MPNST
Cause of Malignant Nerve Sheath Tumors has not been added yet.
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
Treatments of Malignant Nerve Sheath Tumors has not been added yet.
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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CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

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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