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Genetic and Rare Diseases Information Center (GARD)

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Malignant peripheral nerve sheath tumor


Other Names for this Disease
  • MPNST
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Overview



What is a malignant peripheral nerve sheath tumor?


What is a malignant peripheral nerve sheath tumor?

A malignant peripheral nerve sheath tumor (MPNST) is a tumor that develops from nerve tissue.  The first symptom of MPNST is 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][2] and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.[3][4][5]  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]  MPNSTs are quite rare, occurring in 0.001% of the general population.[6]  Approximately 25-50% of MPNSTs are associated with a genetic condition known as neurofibromatosis type 1.[7]
Last updated: 10/18/2011

References
  1. 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. Accessed 10/14/2011.
  2. 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. Accessed 10/14/2011.
  3. 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. Accessed 10/14/2011.
  4. 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. Accessed 10/14/2011.
  5. 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. Accessed 10/15/2011.
  6. 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. Accessed 10/14/2011.
  7. 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. Accessed 10/14/2011.