Malignant peripheral nerve sheath tumor pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marjan Khan M.B.B.S.[2]
Overview
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 a herring bone pattern are characteristic findings of malignant peripheral nerve sheath tumor.[1] The neurofibromatosis type I (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.[2]
Pathogenesis
- Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk.[3]
Genetics
- The genotypic hallmark of NF1 involves mutations to or other loss of the 350 kilobase gene NF1 on the long arm of chromosome 17, which encodes the tumor suppressor protein neurofibromin.
- NF1 inactivation leads to ras hyperactivity and consequent activation of multiple downstream survival and proliferative pathways, including the mitogen-activated protein kinase (MAPK), mammalian target of rapamycin (mTOR), and AKT (Mouse breed AK thymoma, also termed protein kinase B, or PKB) pathways.
- About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with NF1. The lifetime risk of having both of these conditions is at 8–13% while those with only MPNST have a 0.001% in the general population.[4]
- The NF1 gene locus is on chromosome 17q11.2 and the gene product is neurofibromin, acts as a tumour suppressor; inactivation of the gene thus predisposes to tumour development.[5]
Gross Pathology
- Large and firm mass[6]
- Necrosis
- Hemorrhage
Microscopic Pathology
- Cellular[1]
- Usually spindle cells
- Rarely epithelioid
- Nuclear atypia
- Mitoses
- Herring bone pattern
-
![Malignant peripheral nerve sheath tumor[1]](/images/f/fa/Malignant_peripheral_nerve_sheath_tumour.jpg)
Malignant peripheral nerve sheath tumor[1]
![Malignant peripheral nerve sheath tumor[1]](/index.php/File:Malignant_peripheral_nerve_sheath_tumour.jpg)
References
- ↑ 1.0 1.1 1.2 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) - ↑ Panigrahi, S.; Mishra, S.; Das, S.; Dhir, M. (2013). "Primary malignant peripheral nerve sheath tumor at unusual location". Journal of Neurosciences in Rural Practice. 4 (5): 83. doi:10.4103/0976-3147.116480. PMC 3808069. PMID 24174807.
- ↑ 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.
- ↑ Fetsch JF, Laskin WB, Miettinen M (2005). "Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate". Am J Surg Pathol. 29 (12): 1615–24. PMID 16327434.