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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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Your 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?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is the prognosis for malignant peripheral nerve sheath tumors?

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.[1][2]  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).[2]  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.[3]
Last updated: 10/18/2011

References
  • 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.
  • 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.
  • 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.