Malignant peripheral nerve sheath tumour - Revision history

Jensflorian: /* IHC */ update

2017-04-27T09:28:35Z

IHC: update

← Older revision		Revision as of 09:28, 27 April 2017
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	*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>		*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>
			*H3K27me -ve<ref>{{Cite journal \| last1 = Schaefer \| first1 = IM. \| last2 = Fletcher \| first2 = CD. \| last3 = Hornick \| first3 = JL. \| title = Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics. \| journal = Mod Pathol \| volume = 29 \| issue = 1 \| pages = 4-13 \| month = Jan \| year = 2016 \| doi = 10.1038/modpathol.2015.134 \| PMID = 26585554 }}</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>		*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>

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	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 -ve.		*p16 -ve.<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>
	*p27.		*p27.
	*p75NTR +ve (80%).		*p75NTR +ve (80%).
			*EGFR +ve <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>

	==Molecular==		==Molecular==

Jensflorian: /* Microscopic */ update

2016-05-09T10:17:07Z

Microscopic: update

← Older revision		Revision as of 10:17, 9 May 2016
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	*Mitoses.		*Mitoses.
	*+/-Herring bone pattern.		*+/-Herring bone pattern.
	*Perivascular hypercellularity		*Perivascular hypercellularity.
	*Tumor herniation into vascular lumens		*Tumor herniation into vascular lumens.
			*"Pseudocapsule" with tumour invasion of adjacent tissue.

	Notes:		Notes:
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	*Plexiform schwannoma.		*Plexiform schwannoma.
	*[[Malignant triton tumour]].		*[[Malignant triton tumour]].
			**aka. MPNST with mesenchymal differentiation.

	DDx of herring bone:		DDx of herring bone:
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	===Grading===		===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>		*Can be graded histologically, according WHO<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>
			*grade IV: Presence of necrosis (3/4 of all MPNST).
			*grade III: Mitotic count exceeds 4/1 HPF.
			*grade II: nuclear size (<3x of neurofibroma cell), hyperchromatic nuclei.
			**overlap with cellular ("atypical") neurofibroma (DDx).


	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:		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:

Jensflorian: /* General */ update

2016-05-09T10:09:30Z

General: update

← Older revision		Revision as of 10:09, 9 May 2016
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	*Malignant - as the name implies.		*Malignant - as the name implies.
	*Usually assoc. with a peripheral nerve.<ref>{WHOCNS2007}</ref>		*Usually assoc. with a peripheral nerve.<ref>{WHOCNS2007}</ref>
	*May be seen in the context of [[neurofibromatosis type 1]].		*May be seen in the context of [[neurofibromatosis type 1]] or radiation induced.<ref>{{Cite journal \| last1 = Ducatman \| first1 = BS. \| last2 = Scheithauer \| first2 = BW. \| last3 = Piepgras \| first3 = DG. \| last4 = Reiman \| first4 = HM. \| last5 = Ilstrup \| first5 = DM. \| title = Malignant peripheral nerve sheath tumors. A clinicopathologic study of 120 cases. \| journal = Cancer \| volume = 57 \| issue = 10 \| pages = 2006-21 \| month = May \| year = 1986 \| doi = \| PMID = 3082508 }}</ref>
			*Approx 50-70% arise from (mostly plexiform) [[neurofibroma]]s.
			*Usu. adults
			*Approx. 5% of all malignant soft tissue lesions.

	<gallery>		<gallery>

Jensflorian: terminology and missing citation added.

2016-05-09T08:12:46Z

terminology and missing citation added.

← Older revision		Revision as of 08:12, 9 May 2016
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	'''Malignant peripheral nerve sheath tumour''', abbreviated '''MPNST''', is an uncommon malignant tumour of the nerve sheath.		'''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>		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>, '''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> and '''malignant schwannoma''' - these terms are however depreceated.<ref>{WHOCNS2007}</ref>

	==General==		==General==
	*Malignant - as the name implies.		*Malignant - as the name implies.
	*Usually assoc. with a peripheral nerve.{~~{Fact}~~}		*Usually assoc. with a peripheral nerve.<ref>{WHOCNS2007}</ref>
	*May be seen in the context of [[neurofibromatosis type 1]].		*May be seen in the context of [[neurofibromatosis type 1]].

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	==Molecular==		==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>		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><ref>{{Cite journal \| last1 = Hirbe \| first1 = AC. \| last2 = Dahiya \| first2 = S. \| last3 = Miller \| first3 = CA. \| last4 = Li \| first4 = T. \| last5 = Fulton \| first5 = RS. \| last6 = Zhang \| first6 = X. \| last7 = McDonald \| first7 = S. \| last8 = DeSchryver \| first8 = K. \| last9 = Duncavage \| first9 = EJ. \| title = Whole Exome Sequencing Reveals the Order of Genetic Changes during Malignant Transformation and Metastasis in a Single Patient with NF1-plexiform Neurofibroma. \| journal = Clin Cancer Res \| volume = 21 \| issue = 18 \| pages = 4201-11 \| month = Sep \| year = 2015 \| doi = 10.1158/1078-0432.CCR-14-3049 \| PMID = 25925892 }}</ref>
	* Atypical neurofibromas and low-grade MPNST have a common methylation profile and frequent losses of CDKN2A.		* 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).		* A subset of MPNST show loss of trimethylation of histone H3 at lysine 27 (H3K27me3).
			* P53 and beta-spectrin mutations during progression reported.
	==See also==		==See also==
	*[[Peripheral nerve sheath tumours]].		*[[Peripheral nerve sheath tumours]].

Jensflorian: Image, IHC update

2016-05-09T08:05:52Z

Image, IHC update

← Older revision		Revision as of 08:05, 9 May 2016
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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.
	*~~MIB1~~.		*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==

Michael: touch

2015-05-04T05:49:56Z

touch

← Older revision		Revision as of 05:49, 4 May 2015
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	{{ Infobox diagnosis		{{ Infobox diagnosis
	\| Name = {{PAGENAME}}		\| Name = {{PAGENAME}}
	\| Image = Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg		\| Image = Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg
	\| Width =		\| Width =
	\| Caption = MPNST. [[H&E stain]].		\| Caption = MPNST. [[H&E stain]].

Michael: /* Images */

2014-11-01T06:56:25Z

Images

← Older revision		Revision as of 06:56, 1 November 2014
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	Image:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg \| MPNST - intermed. mag. (WC)		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)		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)
	</gallery>		</gallery>
	www:		www:

Michael at 09:58, 7 October 2013

2013-10-07T09:58:18Z

← Older revision		Revision as of 09:58, 7 October 2013
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	\| Width =		\| Width =
	\| Caption = MPNST. [[H&E stain]].		\| Caption = MPNST. [[H&E stain]].
	\| Micro = spindle cell lesion (or very rarely epithelioid) with nuclear atypia, mitotic activity, +/-[[herring bone pattern]]		\| Micro = spindle cell lesion (or very rarely epithelioid lesion) with nuclear atypia, mitotic activity, +/-[[herring bone pattern]]
	\| Subtypes = [[malignant triton tumour]]		\| Subtypes = [[malignant triton tumour]]
	\| LMDDx = [[synovial sarcoma]], [[fibrosarcoma]], cellular [[schwannoma]], plexiform schwannoma		\| LMDDx = [[synovial sarcoma]], [[fibrosarcoma]], cellular [[schwannoma]], plexiform schwannoma

Michael: /* Microscopic */

2013-10-07T09:57:59Z

Microscopic

← Older revision		Revision as of 09:57, 7 October 2013
Line 40:		Line 40:
	Features:		Features:
	*Cellular - usu. spindle cells.		*Cellular - usu. spindle cells.
	**~~Rarely~~ epithelioid.		**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>
	*Nuclear atypia.		*Nuclear atypia.
	*Mitoses.		*Mitoses.
Line 47:		Line 47:
	Notes:		Notes:
	*May be diagnosed in a poorly diff. tumour if patient has [[NF1]].		*May be diagnosed in a poorly diff. tumour if patient has [[NF1]].
	*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>

	DDx:		DDx:

Michael at 09:56, 7 October 2013

2013-10-07T09:56:21Z

← Older revision		Revision as of 09:56, 7 October 2013
Line 4:		Line 4:
	\| Width =		\| Width =
	\| Caption = MPNST. [[H&E stain]].		\| Caption = MPNST. [[H&E stain]].
	\| Micro = ~~spindel~~ cell lesion with nuclear atypia, mitotic activity, +/-[[herring bone pattern]]		\| Micro = spindle cell lesion (or very rarely epithelioid) with nuclear atypia, mitotic activity, +/-[[herring bone pattern]]
	\| Subtypes = [[malignant triton tumour]]		\| Subtypes = [[malignant triton tumour]]
	\| LMDDx = [[synovial sarcoma]], [[fibrosarcoma]], cellular [[schwannoma]], plexiform schwannoma		\| LMDDx = [[synovial sarcoma]], [[fibrosarcoma]], cellular [[schwannoma]], plexiform schwannoma