Malignant peripheral nerve sheath tumor overview: Difference between revisions
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==Overview== | ==Overview== | ||
Malignant peripheral nerve sheath tumor (MPNST) is a form of [[cancer]] of the [[connective tissue]] surrounding [[nerve]]s. 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.<ref>Malignant peripheral nerve sheath tumor. Librepathology 2015. http://librepathology.org/wiki/index.php/Malignant_peripheral_nerve_sheath_tumour</ref> [[Neurofibromatosis type I]] (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.<ref>{{cite journal|last=Ferrari|first=Andrea|author2=Gianni Bisogno |author3=Modesto Carli |title=Management Of Childhood Malignant Peripheral Nerve Sheath Tumor | journal=Pediatric Drugs | date=2007|volume=9|issue=4|pages=239–48|accessdate=5 Mar 2014 | pmid=17705563 | doi=10.2165/00148581-200709040-00005}}</ref> Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and [[schwannoma]].<ref>Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> The incidence of malignant peripheral nerve sheath tumor increases with age.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> Malignant peripheral nerve sheath tumor affects men and women equally.<ref>Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> 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]].<ref name="pmid15177319">{{cite journal| author=Perrin RG, Guha A| title=Malignant peripheral nerve sheath tumors. | journal=Neurosurg Clin N Am | year= 2004 | volume= 15 | issue= 2 | pages= 203-16 | pmid=15177319 | doi=10.1016/j.nec.2004.02.004 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15177319 }} </ref><ref name="pmid12963767">{{cite journal| author=Baehring JM, Betensky RA, Batchelor TT| title=Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. | journal=Neurology | year= 2003 | volume= 61 | issue= 5 | pages= 696-8 | pmid=12963767 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12963767 }} </ref><ref name="pmid15739552">{{cite journal| author=Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG| title=A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center. | journal=J Neurosurg | year= 2005 | volume= 102 | issue= 2 | pages= 246-55 | pmid=15739552 | doi=10.3171/jns.2005.102.2.0246 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15739552 }} </ref> Symptoms of malignant peripheral nerve sheath tumor include [[peripheral edema]], difficulty in moving the extremity, [[pain]], and [[numbness]].<ref name="pmid16029330">{{cite journal| author=Valeyrie-Allanore L, Ismaïli N, Bastuji-Garin S, Zeller J, Wechsler J, Revuz J et al.| title=Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1. | journal=Br J Dermatol | year= 2005 | volume= 153 | issue= 1 | pages= 79-82 | pmid=16029330 | doi=10.1111/j.1365-2133.2005.06558.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16029330 }} </ref> [[MRI]] may be diagnostic of malignant peripheral nerve sheath tumor. Findings on [[MRI]] suggestive of malignant peripheral nerve sheath tumor include isointense on T1 weighted image and low signal on T2 weighted image.<ref name=radio> Schwannoma.Dr Tim Luijkx and Dr Sara Wein et al. Dr Mohammad Taghi Niknejad and Dr Yuranga Weerakkody et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/schwannoma </ref> The predominant therapy for malignant peripheral nerve sheath tumor is [[surgical resection]]. Adjunctive [[chemotherapy]] and [[radiation therapy]] may be required. Common [[complication]] of malignant peripheral nerve sheath tumor is [[metastasis]]. [[Prognosis]] is generally poor.<ref name="pmid12632346">{{cite journal |author=Neville H, Corpron C, Blakely ML, Andrassy R |title=Pediatric neurofibrosarcoma |journal=J. Pediatr. Surg. |volume=38 |issue=3 |pages=343–6; discussion 343–6 |year=2003 |pmid=12632346 |doi=10.1053/jpsu.2003.50105}}</ref> | Malignant peripheral nerve sheath tumor (MPNST) is a form of [[cancer]] of the [[connective tissue]] surrounding [[nerve]]s. 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.<ref name=libre>Malignant peripheral nerve sheath tumor. Librepathology 2015. http://librepathology.org/wiki/index.php/Malignant_peripheral_nerve_sheath_tumour</ref> [[Neurofibromatosis type I]] (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.<ref>{{cite journal|last=Ferrari|first=Andrea|author2=Gianni Bisogno |author3=Modesto Carli |title=Management Of Childhood Malignant Peripheral Nerve Sheath Tumor | journal=Pediatric Drugs | date=2007|volume=9|issue=4|pages=239–48|accessdate=5 Mar 2014 | pmid=17705563 | doi=10.2165/00148581-200709040-00005}}</ref> Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and [[schwannoma]].<ref name=radio>Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> The incidence of malignant peripheral nerve sheath tumor increases with age.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> Malignant peripheral nerve sheath tumor affects men and women equally.<ref name=radio> Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> 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]].<ref name="pmid15177319">{{cite journal| author=Perrin RG, Guha A| title=Malignant peripheral nerve sheath tumors. | journal=Neurosurg Clin N Am | year= 2004 | volume= 15 | issue= 2 | pages= 203-16 | pmid=15177319 | doi=10.1016/j.nec.2004.02.004 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15177319 }} </ref><ref name="pmid12963767">{{cite journal| author=Baehring JM, Betensky RA, Batchelor TT| title=Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. | journal=Neurology | year= 2003 | volume= 61 | issue= 5 | pages= 696-8 | pmid=12963767 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12963767 }} </ref><ref name="pmid15739552">{{cite journal| author=Kim DH, Murovic JA, Tiel RL, Moes G, Kline DG| title=A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center. | journal=J Neurosurg | year= 2005 | volume= 102 | issue= 2 | pages= 246-55 | pmid=15739552 | doi=10.3171/jns.2005.102.2.0246 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15739552 }} </ref> Symptoms of malignant peripheral nerve sheath tumor include [[peripheral edema]], difficulty in moving the extremity, [[pain]], and [[numbness]].<ref name="pmid16029330">{{cite journal| author=Valeyrie-Allanore L, Ismaïli N, Bastuji-Garin S, Zeller J, Wechsler J, Revuz J et al.| title=Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1. | journal=Br J Dermatol | year= 2005 | volume= 153 | issue= 1 | pages= 79-82 | pmid=16029330 | doi=10.1111/j.1365-2133.2005.06558.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16029330 }} </ref> [[MRI]] may be diagnostic of malignant peripheral nerve sheath tumor. Findings on [[MRI]] suggestive of malignant peripheral nerve sheath tumor include isointense on T1 weighted image and low signal on T2 weighted image.<ref name=radio> Schwannoma.Dr Tim Luijkx and Dr Sara Wein et al. Dr Mohammad Taghi Niknejad and Dr Yuranga Weerakkody et al. Radiopaedia.org 2015. http://radiopaedia.org/articles/schwannoma </ref> The predominant therapy for malignant peripheral nerve sheath tumor is [[surgical resection]]. Adjunctive [[chemotherapy]] and [[radiation therapy]] may be required. Common [[complication]] of malignant peripheral nerve sheath tumor is [[metastasis]]. [[Prognosis]] is generally poor.<ref name="pmid12632346">{{cite journal |author=Neville H, Corpron C, Blakely ML, Andrassy R |title=Pediatric neurofibrosarcoma |journal=J. Pediatr. Surg. |volume=38 |issue=3 |pages=343–6; discussion 343–6 |year=2003 |pmid=12632346 |doi=10.1053/jpsu.2003.50105}}</ref> | ||
==Classification== | ==Classification== | ||
| Line 10: | Line 10: | ||
==Pathophysiology== | ==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.<ref>Malignant peripheral nerve sheath tumor. Librepathology 2015. http://librepathology.org/wiki/index.php/Malignant_peripheral_nerve_sheath_tumour</ref> [[Neurofibromatosis type I]] (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.<ref>{{cite journal|last=Ferrari|first=Andrea|author2=Gianni Bisogno |author3=Modesto Carli |title=Management Of Childhood Malignant Peripheral Nerve Sheath Tumor | journal=Pediatric Drugs | date=2007|volume=9|issue=4|pages=239–48|accessdate=5 Mar 2014 | pmid=17705563 | doi=10.2165/00148581-200709040-00005}}</ref> | 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.<ref name=libre>Malignant peripheral nerve sheath tumor. Librepathology 2015. http://librepathology.org/wiki/index.php/Malignant_peripheral_nerve_sheath_tumour</ref> [[Neurofibromatosis type I]] (NF1) gene is involved in the pathogenesis of malignant peripheral nerve sheath tumor.<ref>{{cite journal|last=Ferrari|first=Andrea|author2=Gianni Bisogno |author3=Modesto Carli |title=Management Of Childhood Malignant Peripheral Nerve Sheath Tumor | journal=Pediatric Drugs | date=2007|volume=9|issue=4|pages=239–48|accessdate=5 Mar 2014 | pmid=17705563 | doi=10.2165/00148581-200709040-00005}}</ref> | ||
==Causes== | ==Causes== | ||
| Line 16: | Line 16: | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and [[schwannoma]].<ref>Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> | Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and [[schwannoma]].<ref name=radio> Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> | ||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> The incidence of malignant peripheral nerve sheath tumor increases with age.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> Malignant peripheral nerve sheath tumor affects men and women equally.<ref>Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> | The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> The incidence of malignant peripheral nerve sheath tumor increases with age.<ref name="pmid25130403">{{cite journal| author=Bates JE, Peterson CR, Dhakal S, Giampoli EJ, Constine LS| title=Malignant peripheral nerve sheath tumors (MPNST): a SEER analysis of incidence across the age spectrum and therapeutic interventions in the pediatric population. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 11 | pages= 1955-60 | pmid=25130403 | doi=10.1002/pbc.25149 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25130403 }} </ref> Malignant peripheral nerve sheath tumor affects men and women equally.<ref name=radio> Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> | ||
==Risk Factors== | ==Risk Factors== | ||
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===CT=== | ===CT=== | ||
[[CT scan]] may be diagnostic of malignant peripheral nerve sheath tumor. Finding on [[CT scan]] suggestive of malignant peripheral nerve sheath tumor is a rapid growth [[mass]] with irrefular border.<ref>Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> | [[CT scan]] may be diagnostic of malignant peripheral nerve sheath tumor. Finding on [[CT scan]] suggestive of malignant peripheral nerve sheath tumor is a rapid growth [[mass]] with irrefular border.<ref name=radio> Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour</ref> | ||
===MRI=== | ===MRI=== | ||
Revision as of 15:40, 18 December 2015
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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. 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] Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and schwannoma.[3] The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.[4] The incidence of malignant peripheral nerve sheath tumor increases with age.[4] Malignant peripheral nerve sheath tumor affects men and women equally.[3] 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.[5][6][7] Symptoms of malignant peripheral nerve sheath tumor include peripheral edema, difficulty in moving the extremity, pain, and numbness.[8] MRI may be diagnostic of malignant peripheral nerve sheath tumor. Findings on MRI suggestive of malignant peripheral nerve sheath tumor include isointense on T1 weighted image and low signal on T2 weighted image.[3] The predominant therapy for malignant peripheral nerve sheath tumor is surgical resection. Adjunctive chemotherapy and radiation therapy may be required. Common complication of malignant peripheral nerve sheath tumor is metastasis. Prognosis is generally poor.[9]
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.[10]
Causes
Malignant peripheral nerve sheath tumor may be caused by a mutation on neurofibromatosis type I gene.[11][12]
Differential Diagnosis
Malignant peripheral nerve sheath tumor must be differentiated from neurofibroma and schwannoma.[3]
Epidemiology and Demographics
The incidence of malignant peripheral nerve sheath tumor is approximately 0.1 per 100,000 individuals worldwide.[4] The incidence of malignant peripheral nerve sheath tumor increases with age.[4] Malignant peripheral nerve sheath tumor affects men and women equally.[3]
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.[5][6][7]
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.[13]
Complication and prognosis
Common complication of malignant peripheral nerve sheath tumor is metastasis. Prognosis is generally poor.[9]
Diagnosis
Staging
There is no established system for the staging of malignant peripheral nerve sheath tumor.
Symptoms
Symptoms of malignant peripheral nerve sheath tumor include peripheral edema, difficulty in moving the extremity, pain, and numbness.[8]
Physical Examination
Common physical examination findings of malignant peripheral nerve sheath tumor include edema, paresthesia, and weakness.
Laboratory Findings
There are no laboratory findings associated with malignant peripheral nerve sheath tumor.
X Ray
There are no X-ray findings associated with malignant peripheral nerve sheath tumor.
CT
CT scan may be diagnostic of malignant peripheral nerve sheath tumor. Finding on CT scan suggestive of malignant peripheral nerve sheath tumor is a rapid growth mass with irrefular border.[3]
MRI
MRI may be diagnostic of malignant peripheral nerve sheath tumor. Findings on MRI suggestive of malignant peripheral nerve sheath tumor include isointense on T1 weighted image and low signal on T2 weighted image.[3]
Ultrasound
There are no ultrasound findings associated with malignant peripheral nerve sheath tumor.
Other Imaging Findings
Scintigraphy may be diagnostic of malignant peripheral nerve sheath tumor. Finding on Gallium67 scintigraphy suggestive of malignant peripheral nerve sheath tumor is higher uptake.[3]
Other Diagnostic Studies
There are no other diagnostic study findings associated with malignant peripheral nerve sheath tumor.
Biopsy
Biopsy is helpful in the diagnosis of schwannoma.
Treatment
Medical Therapy
The predominant therapy for malignant peripheral nerve sheath tumor is surgical resection. Adjunctive chemotherapy and radiation therapy may be required.
Surgery
Surgery is the mainstay of treatment for malignant peripheral nerve sheath tumor.
Primary Prevention
There is no established method for prevention of malignant peripheral nerve sheath tumor.
Secondary Prevention
There are no secondary preventive measures available for malignant peripheral nerve sheath tumor.
References
- ↑ 1.0 1.1 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) - ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Malignant peripheral nerve sheath tumor. Radiopaedia 2015. http://radiopaedia.org/articles/malignant-peripheral-nerve-sheath-tumour
- ↑ 4.0 4.1 4.2 4.3 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.
- ↑ 5.0 5.1 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.
- ↑ 6.0 6.1 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.
- ↑ 7.0 7.1 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.
- ↑ 8.0 8.1 Valeyrie-Allanore L, Ismaïli N, Bastuji-Garin S, Zeller J, Wechsler J, Revuz J; et al. (2005). "Symptoms associated with malignancy of peripheral nerve sheath tumours: a retrospective study of 69 patients with neurofibromatosis 1". Br J Dermatol. 153 (1): 79–82. doi:10.1111/j.1365-2133.2005.06558.x. PMID 16029330.
- ↑ 9.0 9.1 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.
- ↑ 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. U.S. Preventive Service Task Force (USPSTF) 2015. http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=Malignant+peripheral+nerve+sheath+tumor