Difference between revisions of "Malignant peripheral nerve sheath tumour"

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#redirect [[Peripheral nerve sheath tumours#Malignant peripheral nerve sheath tumour]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      =
| Width      =
| Caption    =
| Micro      =
| Subtypes  =
| LMDDx      = [[synovial sarcoma]], [[fibrosarcoma]], cellular [[schwannoma]], plexiform schwannoma, [[malignant triton tumour]].
| Stains    =
| IHC        =
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence =
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
}}
'''Malignant peripheral nerve sheath tumour''', abbreviated ''MPNST'', is an uncommon malignant tumour of the nerve sheath.
 
It is also known ''neurofibrosarcoma''<ref name=pmid21317712>{{Cite journal  | last1 = Mills | first1 = AM. | last2 = Karamchandani | first2 = JR. | last3 = Vogel | first3 = H. | last4 = Longacre | first4 = TA. | title = Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes. | journal = Am J Surg Pathol | volume = 35 | issue = 3 | pages = 404-12 | month = Mar | year = 2011 | doi = 10.1097/PAS.0b013e318208f72e | PMID = 21317712 }}</ref> and '''neurogenic sarcoma.<ref name=pmid23139572>{{Cite journal  | last1 = Sham | first1 = ME. | last2 = Ghorpade | first2 = A. | last3 = Shetty | first3 = S. | last4 = Hari | first4 = . | last5 = Vinay | first5 = . | title = Malignant peripheral nerve cell tumour. | journal = J Maxillofac Oral Surg | volume = 9 | issue = 1 | pages = 68-71 | month = Mar | year = 2010 | doi = 10.1007/s12663-010-0019-6 | PMID = 23139572 }}</ref>
 
==General==
*Malignant - as the name implies.
*Usually assoc. with a peripheral nerve.{{Fact}}
*May be seen in the context of [[neurofibromatosis type 1]].
 
==Microscopic==
Features:
*Cellular.
*Nuclear atypia.
*Mitoses.
*+/-Herring bone pattern.
 
Notes:
*May be diagnosed in a poorly diff. tumour if patient has [[NF1]].
 
DDx:
*Cellular [[schwannoma]].
*Plexiform schwannoma.
*[[Malignant triton tumour]].
 
DDx of herring bone:
*MPNST.
*[[Synovial sarcoma]].
*[[Fibrosarcoma]].
 
===Images===
<gallery>
Image:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg | MPNST - intermed. mag. (WC)
Image:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg | MPNST - high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case112/micro.html MPNST - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case406.html MPNST - case 2 - several images (upmc.edu)].
 
===Grading===
*Can be graded histologically,<ref name=pmid14508395>{{cite journal |author=Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH |title=Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions |journal=Am. J. Surg. Pathol. |volume=27 |issue=10 |pages=1337–45 |year=2003 |month=October |pmid=14508395 |doi= |url=}}</ref> and this is prognostic.<ref name=pmid16923196>{{cite journal |author=Kar M, Deo SV, Shukla NK, ''et al.'' |title=Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases |journal=World J Surg Oncol |volume=4 |issue= |pages=55 |year=2006 |pmid=16923196 |pmc=1560134 |doi=10.1186/1477-7819-4-55 |url=}}</ref>
 
Sarcoma grading system<ref name=pmid6693192>{{cite journal |author=Trojani M, Contesso G, Coindre JM, ''et al.'' |title=Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system |journal=Int. J. Cancer |volume=33 |issue=1 |pages=37–42 |year=1984 |month=January |pmid=6693192 |doi= |url=}}</ref> - based on:
*Tumour differentiation.
*Mitotic rate.
*[[Necrosis]].
 
==IHC==
Features:<ref name=pmid18636017>{{cite journal |author=Nonaka D, Chiriboga L, Rubin BP |title=Sox10: a pan-schwannian and melanocytic marker |journal=Am. J. Surg. Pathol. |volume=32 |issue=9 |pages=1291–8 |year=2008 |month=September |pmid=18636017 |doi=10.1097/PAS.0b013e3181658c14 |url=}}</ref>
*S-100 +ve ~ 30% of tumours.
*SOX10 +ve ~ 50% of tumours.
 
Others:<ref name=pmid14508395>{{cite journal |author=Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH |title=Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions |journal=Am. J. Surg. Pathol. |volume=27 |issue=10 |pages=1337–45 |year=2003 |month=October |pmid=14508395 |doi= |url=}}</ref>
*p53.
*p16.
*p27.
*MIB1.
 
==See also==
*[[Peripheral nerve sheath tumours]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Peripheral nerve sheath tumours]]

Revision as of 05:50, 25 September 2013

Malignant peripheral nerve sheath tumour
Diagnosis in short
LM DDx synovial sarcoma, fibrosarcoma, cellular schwannoma, plexiform schwannoma, malignant triton tumour.
Site soft tissue

Malignant peripheral nerve sheath tumour, abbreviated MPNST, is an uncommon malignant tumour of the nerve sheath.

It is also known neurofibrosarcoma[1] and neurogenic sarcoma.[2]

General

Microscopic

Features:

  • Cellular.
  • Nuclear atypia.
  • Mitoses.
  • +/-Herring bone pattern.

Notes:

  • May be diagnosed in a poorly diff. tumour if patient has NF1.

DDx:

DDx of herring bone:

Images

  • MPNST - intermed. mag. (WC)

  • MPNST - high mag. (WC)

www:

Grading

  • Can be graded histologically,[3] and this is prognostic.[4]

Sarcoma grading system[5] - based on:

  • Tumour differentiation.
  • Mitotic rate.
  • Necrosis.

IHC

Features:[6]

  • S-100 +ve ~ 30% of tumours.
  • SOX10 +ve ~ 50% of tumours.

Others:[3]

  • p53.
  • p16.
  • p27.
  • MIB1.

See also

References

  1. ↑ Mills, AM.; Karamchandani, JR.; Vogel, H.; Longacre, TA. (Mar 2011). "Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes.". Am J Surg Pathol 35 (3): 404-12. doi:10.1097/PAS.0b013e318208f72e. PMID 21317712.
  2. ↑ Sham, ME.; Ghorpade, A.; Shetty, S.; Hari, .; Vinay, . (Mar 2010). "Malignant peripheral nerve cell tumour.". J Maxillofac Oral Surg 9 (1): 68-71. doi:10.1007/s12663-010-0019-6. PMID 23139572.
  3. ↑ 3.0 3.1 Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH (October 2003). "Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions". Am. J. Surg. Pathol. 27 (10): 1337–45. PMID 14508395.
  4. ↑ Kar M, Deo SV, Shukla NK, et al. (2006). "Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases". World J Surg Oncol 4: 55. doi:10.1186/1477-7819-4-55. PMC 1560134. PMID 16923196. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1560134/.
  5. ↑ Trojani M, Contesso G, Coindre JM, et al. (January 1984). "Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system". Int. J. Cancer 33 (1): 37–42. PMID 6693192.
  6. ↑ Nonaka D, Chiriboga L, Rubin BP (September 2008). "Sox10: a pan-schwannian and melanocytic marker". Am. J. Surg. Pathol. 32 (9): 1291–8. doi:10.1097/PAS.0b013e3181658c14. PMID 18636017.
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