Malignant Peripheral Nerve Sheath Tumor (MPNST) malady

NIH Rare Diseases:⁴³ 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.  mpnst is considered an aggressive tumor because there is up to a 65% chance of the tumor regrowing after surgery (a recurrence), and approximately 40% chance of spreading to distant parts of the body (a metastasis), most commonly to the lung.  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.  mpnsts are quite rare, occurring in 0.001% of the general population.  approximately 25-50% of mpnsts are associated with a genetic condition known as neurofibromatosis type 1. last updated: 10/18/2011

MalaCards: Malignant Peripheral Nerve Sheath Tumor, also known as MPNST, is related to neurofibromatosis and plexiform neurofibroma. An important gene associated with Malignant Peripheral Nerve Sheath Tumor is S100B (S100 calcium binding protein B), and among its related pathways are GPCR Pathway and Glioblastoma Multiforme. The compounds estrogen and glucose have been mentioned in the context of this disorder. Related mouse phenotypes are limbs/digits/tail and embryogenesis.

Wikipedia:⁶⁴ A malignant peripheral nerve sheath tumor (MPNST) (also known as \"Malignant schwannoma,\"... more...

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Disease Ontology8 DOID:5940 MeSH35 D018317 SNOMED-CT57 19897006, 404037002, 134324009

NCIt40 C3798 UMLS61 C0751690

Search GEO for disease gene expression data for Malignant Peripheral Nerve Sheath Tumor.