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Malignant peripheral nerve sheath tumour (view source)
Revision as of 08:05, 9 May 2016
, 08:05, 9 May 2016Image, IHC update
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*Usually assoc. with a peripheral nerve.{{Fact}} | *Usually assoc. with a peripheral nerve.{{Fact}} | ||
*May be seen in the context of [[neurofibromatosis type 1]]. | *May be seen in the context of [[neurofibromatosis type 1]]. | ||
<gallery> | |||
File:MPNST.PNG | MPNST in a NF1 case (WC/Filip em). | |||
File:MPNST Pathology gross.jpg | MPNST gross pathology (Flickr/drbloodmoney). | |||
</gallery> | |||
==Microscopic== | ==Microscopic== | ||
Features: | Features:<ref>{{Cite journal | last1 = Pekmezci | first1 = M. | last2 = Reuss | first2 = DE. | last3 = Hirbe | first3 = AC. | last4 = Dahiya | first4 = S. | last5 = Gutmann | first5 = DH. | last6 = von Deimling | first6 = A. | last7 = Horvai | first7 = AE. | last8 = Perry | first8 = A. | title = Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas. | journal = Mod Pathol | volume = 28 | issue = 2 | pages = 187-200 | month = Feb | year = 2015 | doi = 10.1038/modpathol.2014.109 | PMID = 25189642 }}</ref> | ||
*Cellular - usu. spindle cells. | *Cellular - usu. spindle cells. | ||
**Very rarely epithelioid.<ref name=pmid22082606>{{Cite journal | last1 = Carter | first1 = JM. | last2 = O'Hara | first2 = C. | last3 = Dundas | first3 = G. | last4 = Gilchrist | first4 = D. | last5 = Collins | first5 = MS. | last6 = Eaton | first6 = K. | last7 = Judkins | first7 = AR. | last8 = Biegel | first8 = JA. | last9 = Folpe | first9 = AL. | title = Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with neuroblastoma-like schwannomatosis and a novel germline SMARCB1 mutation. | journal = Am J Surg Pathol | volume = 36 | issue = 1 | pages = 154-60 | month = Jan | year = 2012 | doi = 10.1097/PAS.0b013e3182380802 | PMID = 22082606 }}</ref> | **Very rarely epithelioid.<ref name=pmid22082606>{{Cite journal | last1 = Carter | first1 = JM. | last2 = O'Hara | first2 = C. | last3 = Dundas | first3 = G. | last4 = Gilchrist | first4 = D. | last5 = Collins | first5 = MS. | last6 = Eaton | first6 = K. | last7 = Judkins | first7 = AR. | last8 = Biegel | first8 = JA. | last9 = Folpe | first9 = AL. | title = Epithelioid malignant peripheral nerve sheath tumor arising in a schwannoma, in a patient with neuroblastoma-like schwannomatosis and a novel germline SMARCB1 mutation. | journal = Am J Surg Pathol | volume = 36 | issue = 1 | pages = 154-60 | month = Jan | year = 2012 | doi = 10.1097/PAS.0b013e3182380802 | PMID = 22082606 }}</ref> | ||
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*Mitoses. | *Mitoses. | ||
*+/-Herring bone pattern. | *+/-Herring bone pattern. | ||
*Perivascular hypercellularity | |||
*Tumor herniation into vascular lumens | |||
Notes: | Notes: | ||
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Image:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg | MPNST - high mag. (WC) | Image:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg | MPNST - high mag. (WC) | ||
Image:Malignant_peripheral_nerve_sheath_tumour_-_very_high_mag.jpg | MPNST - very high mag. (WC) | Image:Malignant_peripheral_nerve_sheath_tumour_-_very_high_mag.jpg | MPNST - very high mag. (WC) | ||
File:MPNST chondroid differentiation.jpg | MPNST with chondroid dedifferentiation (Triton tumor). (WC) | |||
</gallery> | </gallery> | ||
www: | www: | ||
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*S-100 +ve ~ 30% of tumours. | *S-100 +ve ~ 30% of tumours. | ||
*SOX10 +ve ~ 50% of tumours. | *SOX10 +ve ~ 50% of tumours. | ||
*Neurofibromin (NFC) -ve (88% in NF1, 43% sporadic MPNST)<ref>{{Cite journal | last1 = Reuss | first1 = DE. | last2 = Habel | first2 = A. | last3 = Hagenlocher | first3 = C. | last4 = Mucha | first4 = J. | last5 = Ackermann | first5 = U. | last6 = Tessmer | first6 = C. | last7 = Meyer | first7 = J. | last8 = Capper | first8 = D. | last9 = Moldenhauer | first9 = G. | title = Neurofibromin specific antibody differentiates malignant peripheral nerve sheath tumors (MPNST) from other spindle cell neoplasms. | journal = Acta Neuropathol | volume = 127 | issue = 4 | pages = 565-72 | month = Apr | year = 2014 | doi = 10.1007/s00401-014-1246-6 | PMID = 24464231 }}</ref> | |||
*MIB-1 ≥20% is highly predictive of malignant peripheral nerve sheath tumor (87% sensitivity and 96% specificity).<ref>{{Cite journal | last1 = Pekmezci | first1 = M. | last2 = Reuss | first2 = DE. | last3 = Hirbe | first3 = AC. | last4 = Dahiya | first4 = S. | last5 = Gutmann | first5 = DH. | last6 = von Deimling | first6 = A. | last7 = Horvai | first7 = AE. | last8 = Perry | first8 = A. | title = Morphologic and immunohistochemical features of malignant peripheral nerve sheath tumors and cellular schwannomas. | journal = Mod Pathol | volume = 28 | issue = 2 | pages = 187-200 | month = Feb | year = 2015 | doi = 10.1038/modpathol.2014.109 | PMID = 25189642 }}</ref> | |||
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> | 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. | *p53. | ||
*p16. | *p16 -ve. | ||
*p27. | *p27. | ||
* | *p75NTR +ve (80%). | ||
==Molecular== | |||
Features:<ref>{{Cite journal | last1 = Röhrich | first1 = M. | last2 = Koelsche | first2 = C. | last3 = Schrimpf | first3 = D. | last4 = Capper | first4 = D. | last5 = Sahm | first5 = F. | last6 = Kratz | first6 = A. | last7 = Reuss | first7 = J. | last8 = Hovestadt | first8 = V. | last9 = Jones | first9 = DT. | title = Methylation-based classification of benign and malignant peripheral nerve sheath tumors. | journal = Acta Neuropathol | volume = | issue = | pages = | month = Feb | year = 2016 | doi = 10.1007/s00401-016-1540-6 | PMID = 26857854 }}</ref> | |||
* Atypical neurofibromas and low-grade MPNST have a common methylation profile and frequent losses of CDKN2A. | |||
* A subset of MPNST show loss of trimethylation of histone H3 at lysine 27 (H3K27me3). | |||
==See also== | ==See also== | ||