Malignant peripheral nerve sheath tumor

Title

Other Names:

MPNST; Malignant neurilemmoma; Malignant neurofibroma; MPNST; Malignant neurilemmoma; Malignant neurofibroma; Malignant schwannoma; Neurofibrosarcoma; Neurogenic sarcoma See More

Categories:

Rare Cancers

This disease is grouped under:

Tumor of cranial and spinal nerves

Summary Summary

A malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops in the protective lining that covers nerves.^[1]^[2]^[3] The first symptom of MPNST is often a lump or mass that increases in size, sometimes causing pain or a tingling sensation.^[1] MPNST is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence),^[1]^[4] and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.^[5]^[6]^[7] Treatment of MPNST begins with surgery to remove as much of the tumor as possible. Radiation therapy may be used to decrease the chance of a recurrence. Chemotherapy might be used if the whole tumor cannot be removed during surgery, or to treat a metastasis.^[1]^[2] MPNSTs are quite rare, occurring in 0.001% of the general population.^[8] Approximately 25-50% of MPNSTs are associated with a genetic condition known as neurofibromatosis type 1.^[9]

Last updated: 8/18/2016

Prognosis Prognosis

Malignant peripheral nerve sheath tumors (MPNST) are aggressive tumors. The chance of surviving a diagnosis of MPNST depends on the size and location of the tumor; people who have a small tumor tend to survive longer than those with a large tumor, and people with a tumor in the arms or legs tend to survive longer those with a tumor in the head and neck regions.^[8]^[9] Also, MPNSTs that are treated when they first occur have a better prognosis than when the tumor has regrown after initial treatments (recurred) or spread to distant parts of the body (metastasized).^[9] One study of 140 patients found that 26% of individuals diagnosed with MPNST were living 10 years after the initial diagnosis. Of those patients who developed a metastasis, 8% were living 10 years after the initial diagnosis.^[6]

Last updated: 8/18/2016

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Related Diseases Related Diseases

Related diseases are conditions that have similar signs and symptoms. A health care provider may consider these conditions in the table below when making a diagnosis. Please note that the table may not include all the possible conditions related to this disease.

Conditions with similar signs and symptoms from Orphanet
Other malignant neoplasms to include in the differential diagnosis include synovial sarcoma, fibrosarcoma, undifferentiated pleomorphic sarcoma, angiosarcoma (see these terms), melanoma, and myoepithelial tumors. Visit the Orphanet disease page for more information.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Malignant peripheral nerve sheath tumor. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Sarcoma Foundation of America
PO Box 98160
Washington, DC 20090-8160
Telephone: +1-301-253-8687
Fax: +1-301-253-8690
E-mail: info@curesarcoma.org
Website: https://www.curesarcoma.org/
Sarcoma UK
49-51 East Road
London, N1 6AH United Kingdom
Toll-free: 0808 801 0401
Telephone: 020 7250 8271
E-mail: info@sarcoma.org.uk
Website: https://sarcoma.org.uk/

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

Mayo Clinic has an information page on Malignant peripheral nerve sheath tumor.
The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
The Sarcoma Foundation of America provides information about Malignant peripheral nerve sheath tumor. Click on the link to open the page for this topic.
Sarcoma UK has an information page about Malignant peripheral nerve sheath tumor. Click on the link to view this page.

In-Depth Information

The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Malignant peripheral nerve sheath tumor. Click on the link to view a sample search on this topic.

News & Events News & Events

News

NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community
May 22, 2020
NCATS Translational Approach Addresses COVID-19
May 21, 2020

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

I was diagnosed with a malignant peripheral nerve sheath tumor in my right thigh. The tumor was taken out surgically. Surgery was followed by 30 sittings of radiation therapy. Several months later, a lump was felt on top of the scar from the first surgery and tests confirmed the lump was the same kind of tumor. Again, the tumor has been removed surgically and chemotherapy is needed. Other tests suggest the tumor has spread to the lungs. What is the prognosis for this type of tumor? See answer

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References References

Grobmyer SR, Reith JD, Shahlaee A, Bush CH, Hochwald SN. Malignant Peripheral Nerve Sheath Tumor: molecular pathogenesis and current management considerations. Journal of Surgical Oncology. 2008; 97:340-349. http://www.ncbi.nlm.nih.gov/pubmed/18286466.
Malignant peripheral nerve sheath tumour (MPNST). Sarcoma UK. August 2015; https://sarcoma.org.uk/sarcoma-types/malignant-peripheral-nerve-sheath-tumour-mpnst#toc-8.
Malignant peripheral nerve sheath tumors. Mayo Clinic. September 3, 2014; http://www.mayoclinic.org/diseases-conditions/malignant-peripheral-nerve-sheath-tumors/basics/definition/con-20035841.
Katz D, Lazar A, Lev D. Malignant peripheral nerve sheath tumour (MPNST): the clinical implications of cellular signalling pathways. Expert Reviews in Molecular Medicine. 2009; 11:e30. http://www.ncbi.nlm.nih.gov/pubmed/19835664.
Stark AM, Buhl R, Hugo HH, Mehdorn HM. Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochirurgica. 2001; 143:357-363. http://www.ncbi.nlm.nih.gov/pubmed/11437289.
Zou C, Smith KD, Liu J, Lahat G, Myers S, Wang WL, Zhang W, McCutcheon IE, Slopis JM, Lazar AJ, Pollock RE, Lev D. Clinical, pathological, and molecular variables predictive of malignant peripheral nerve sheath tumor outcome. Annals of Surgery. 2009; 249:1014-1022. http://www.ncbi.nlm.nih.gov/pubmed/19474676.
Wong WW, Hirose T, Scheithauer BW, Schild SE, Gunderson LL. Malignant peripheral nerve sheath tumor: analysis of treatment outcome. 1998; 42:351-360. http://www.ncbi.nlm.nih.gov/pubmed/9788415.
Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM. Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. Cancer. 1986; 57:2006-2021. http://www.ncbi.nlm.nih.gov/pubmed/3082508.
Anghileri M, Miceli R, Fiore M, Mariani L, Ferrari A, Mussi C, Lozza L, Collini P, Olmi P, Casali PG, Pilotti S, Gronchi A. Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution. Cancer. 2006; 107:1065-1074. http://www.ncbi.nlm.nih.gov/pubmed/16881077.