Malignant peripheral nerve sheath tumor overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding nerves.
Classification
There is no classification system established for malignant peripheral nerve sheath tumor.
Pathophysiology
On gross pathology, a large firm mass with necrosis and hemorrhage is the characteristic finding of malignant peripheral nerve sheath tumor. On microscopic histopathological analysis, spindle cells, nuclear atypia, mitoses, and herring bone pattern are characteristic findings of malignant peripheral nerve sheath tumor.[1] Neurofibromatosis type I (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.[2]
Causes
Malignant peripheral nerve sheath tumor may be caused by a mutation on neurofibromatosis type I gene.[3][4]
Differential Diagnosis
Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and schwannoma.[5]
References
- ↑ Malignant peripheral nerve sheath tumor. Librepathology 2015. http://librepathology.org/wiki/index.php/Malignant_peripheral_nerve_sheath_tumour
- ↑ Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
|access-date=requires|url=(help) - ↑ Albright, A (2008). Principles and practice of pediatric neurosurgery. New York: Thieme. ISBN 1588903958.
- ↑ Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
|access-date=requires|url=(help) - ↑ Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour