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]
Epidemiology and Demographics
The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.[6] The incidence of malignant peripheral nerve sheath tumor increases with age.[6] Malignant peripheral nerve sheath tumor affects men and women equally.[7]
Risk Factors
Common risk factors in the development of malignant peripheral nerve sheath tumor are pre-existing plexiform neurofibromas, pre-existing perineuriomas, radiation therapy, and neurofibromatosis type 1.[8][9][10]
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Malignant peripheral nerve sheath tumor.[11]
Complication and prognosis
Common complication of malignant peripheral nerve sheath tumor is metastasis. Prognosis is generally poor.[12]
Diagnosis
Staging
There is no established system for the staging of malignant peripheral nerve sheath tumor.
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
- ↑ 6.0 6.1 Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS (2014). "Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population". Pediatr Blood Cancer. 61 (11): 1955–60. doi:10.1002/pbc.25149. PMID 25130403.
- ↑ Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour
- ↑ Perrin RG, Guha A (2004). "Malignant peripheral nerve sheath tumors". Neurosurg Clin N Am. 15 (2): 203–16. doi:10.1016/j.nec.2004.02.004. PMID 15177319.
- ↑ Baehring JM, Betensky RA, Batchelor TT (2003). "Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment". Neurology. 61 (5): 696–8. PMID 12963767.
- ↑ Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG (2005). "A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center". J Neurosurg. 102 (2): 246–55. doi:10.3171/jns.2005.102.2.0246. PMID 15739552.
- ↑ Malignant peripheral nerve sheath tumor. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Malignant+peripheral+nerve+sheath+tumor
- ↑ Neville H, Corpron C, Blakely ML, Andrassy R (2003). "Pediatric neurofibrosarcoma". J. Pediatr. Surg. 38 (3): 343–6, discussion 343–6. doi:10.1053/jpsu.2003.50105. PMID 12632346.