STBT5:Solitary fibrous tumour - Revision history

Richard.Glen: Removed redirect to CNS5:Solitary fibrous tumour

2026-02-03T18:38:38Z

Removed redirect to CNS5:Solitary fibrous tumour

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Richard.Glen: Redirected page to CNS5:Solitary fibrous tumour

2026-02-03T18:30:43Z

Redirected page to CNS5:Solitary fibrous tumour

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Kathleen.Schieffer at 18:07, 18 December 2025

2025-12-18T18:07:24Z

← Older revision		Revision as of 13:07, 18 December 2025
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	[[STBT5:Table_of_Contents\|Soft Tissue and Bone Tumours (Who Classification, 5th ed.)]]		[[STBT5:Table_of_Contents\|Soft Tissue and Bone Tumours (Who Classification, 5th ed.)]]

	~~{{Under Construction}}~~
	==Primary Author(s)*==		==Primary Author(s)*==
	Yiting Li MD, Reba Daniel MD, and Shashi Shetty PhD		Yiting Li MD, Reba Daniel MD, and Shashi Shetty PhD

Yiting.Li: add name credentials, modify genetic alteration column of gene mutations (tp53)

2025-12-18T15:00:03Z

add name credentials, modify genetic alteration column of gene mutations (tp53)

← Older revision		Revision as of 10:00, 18 December 2025
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	{{Under Construction}}		{{Under Construction}}
	==Primary Author(s)*==		==Primary Author(s)*==
	Yiting Li, Reba Daniel, and Shashi Shetty		Yiting Li MD, Reba Daniel MD, and Shashi Shetty PhD
	==WHO Classification of Disease==		==WHO Classification of Disease==

Line 113:		Line 113:
	\|''TP53''		\|''TP53''
	<br />		<br />
	\|Missense or ~~frame-shift~~ mutation ~~in exon 5 or exon 6~~ <ref name=":3" />		\|Missense or frameshift mutation<ref name=":3" />
	\|Tumor Supressor Gene		\|Tumor Supressor Gene
	\|Common		\|Common

Kathleen.Schieffer at 13:24, 18 December 2025

2025-12-18T13:24:47Z

← Older revision		Revision as of 08:24, 18 December 2025
Line 5:		Line 5:
	{{Under Construction}}		{{Under Construction}}
	==Primary Author(s)*==		==Primary Author(s)*==
	Yiting Li, Reba Daniel and Shashi Shetty		Yiting Li, Reba Daniel, and Shashi Shetty
	==WHO Classification of Disease==		==WHO Classification of Disease==

Line 53:		Line 53:
	\|Common		\|Common
	\|D		\|D
	\|Y (WHO)		\|Yes (WHO)
	\|''NAB2''::''STAT6'' gene fusions are pathognomonic for SFT. Many different breakpoints in the exons and introns are associated with this fusion. The most common fusion variants: ''NAB2''ex4-''STAT6''ex2; ''NAB2''ex6-''STAT6''ex16/17. <ref name=":4" /><ref name=":5" /> Antiangiogenic therapy showed activity in advanced and progressive SFT<ref>{{Cite journal\|last=de Bernardi\|first=Axel\|last2=Dufresne\|first2=Armelle\|last3=Mishellany\|first3=Florence\|last4=Blay\|first4=Jean-Yves\|last5=Ray-Coquard\|first5=Isabelle\|last6=Brahmi\|first6=Mehdi\|date=2022-02-20\|title=Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond\|url=https://www.mdpi.com/2072-6694/14/4/1064\|journal=Cancers\|language=en\|volume=14\|issue=4\|pages=1064\|doi=10.3390/cancers14041064\|issn=2072-6694}}</ref>		\|''NAB2''::''STAT6'' gene fusions are pathognomonic for SFT. Many different breakpoints in the exons and introns are associated with this fusion. The most common fusion variants: ''NAB2''ex4::''STAT6''ex2; ''NAB2''ex6::''STAT6''ex16/17. <ref name=":4" /><ref name=":5" /> Antiangiogenic therapy showed activity in advanced and progressive SFT<ref>{{Cite journal\|last=de Bernardi\|first=Axel\|last2=Dufresne\|first2=Armelle\|last3=Mishellany\|first3=Florence\|last4=Blay\|first4=Jean-Yves\|last5=Ray-Coquard\|first5=Isabelle\|last6=Brahmi\|first6=Mehdi\|date=2022-02-20\|title=Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond\|url=https://www.mdpi.com/2072-6694/14/4/1064\|journal=Cancers\|language=en\|volume=14\|issue=4\|pages=1064\|doi=10.3390/cancers14041064\|issn=2072-6694}}</ref>
	\|}		\|}

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	\|}		\|}
	==Gene Mutations (SNV/INDEL)==		==Gene Mutations (SNV/INDEL)==
	There are multiple genes with single nucleotide variations that have been reported only in metastatic solitary fibrous tumor tissues including TP53 and APAF1.<ref name=":1">{{Cite journal\|last=Park\|first=Hyung Kyu\|last2=Yu\|first2=Dan Bi\|last3=Sung\|first3=Minjung\|last4=Oh\|first4=Ensel\|last5=Kim\|first5=Mingi\|last6=Song\|first6=Ji-Young\|last7=Lee\|first7=Mi-Sook\|last8=Jung\|first8=Kyungsoo\|last9=Noh\|first9=Ka-Won\|date=2019\|title=Molecular changes in solitary fibrous tumor progression\|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6746689/\|journal=Journal of Molecular Medicine (Berlin, Germany)\|volume=97\|issue=10\|pages=1413–1425\|doi=10.1007/s00109-019-01815-8\|issn=0946-2716\|pmc=6746689\|pmid=31321477}}</ref>		There are multiple genes with single nucleotide variations that have been reported only in metastatic solitary fibrous tumor tissues including ''TP53'' and ''APAF1''.<ref name=":1">{{Cite journal\|last=Park\|first=Hyung Kyu\|last2=Yu\|first2=Dan Bi\|last3=Sung\|first3=Minjung\|last4=Oh\|first4=Ensel\|last5=Kim\|first5=Mingi\|last6=Song\|first6=Ji-Young\|last7=Lee\|first7=Mi-Sook\|last8=Jung\|first8=Kyungsoo\|last9=Noh\|first9=Ka-Won\|date=2019\|title=Molecular changes in solitary fibrous tumor progression\|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6746689/\|journal=Journal of Molecular Medicine (Berlin, Germany)\|volume=97\|issue=10\|pages=1413–1425\|doi=10.1007/s00109-019-01815-8\|issn=0946-2716\|pmc=6746689\|pmid=31321477}}</ref>
	{\| class="wikitable sortable"		{\| class="wikitable sortable"
	\|-		\|-
Line 109:		Line 109:
	\|P		\|P
	\|No		\|No
	\|TERT promoter mutations were more frequent in tumors with higher risk of metastasis, have a significant association with malignant SFTs, and may identify intermediate-risk tumors with poorer prognosis.<ref name=":0" /><ref name=":1" /><ref name=":3">{{Cite journal\|last=Bahrami\|first=Armita\|last2=Lee\|first2=Seungjae\|last3=Schaefer\|first3=Inga-Marie\|last4=Boland\|first4=Jennifer M\|last5=Patton\|first5=Kurt T\|last6=Pounds\|first6=Stanley\|last7=Fletcher\|first7=Christopher D\|date=2016-12\|title=TERT promoter mutations and prognosis in solitary fibrous tumor\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222023328\|journal=Modern Pathology\|language=en\|volume=29\|issue=12\|pages=1511–1522\|doi=10.1038/modpathol.2016.126}}</ref><ref>{{Cite journal\|last=Killela\|first=Patrick J.\|last2=Reitman\|first2=Zachary J.\|last3=Jiao\|first3=Yuchen\|last4=Bettegowda\|first4=Chetan\|last5=Agrawal\|first5=Nishant\|last6=Diaz\|first6=Luis A.\|last7=Friedman\|first7=Allan H.\|last8=Friedman\|first8=Henry\|last9=Gallia\|first9=Gary L.\|date=2013-04-09\|title=TERT promoter mutations occur frequently in gliomas and a subset of tumors derived from cells with low rates of self-renewal\|url=https://pnas.org/doi/full/10.1073/pnas.1303607110\|journal=Proceedings of the National Academy of Sciences\|language=en\|volume=110\|issue=15\|pages=6021–6026\|doi=10.1073/pnas.1303607110\|issn=0027-8424\|pmc=PMC3625331\|pmid=23530248}}</ref><ref>{{Cite journal\|last=Koelsche\|first=Christian\|last2=Renner\|first2=Marcus\|last3=Hartmann\|first3=Wolfgang\|last4=Brandt\|first4=Regine\|last5=Lehner\|first5=Burkhard\|last6=Waldburger\|first6=Nina\|last7=Alldinger\|first7=Ingo\|last8=Schmitt\|first8=Thomas\|last9=Egerer\|first9=Gerlinde\|date=2014-12\|title=TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities\|url=https://jeccr.biomedcentral.com/articles/10.1186/1756-9966-33-33\|journal=Journal of Experimental & Clinical Cancer Research\|language=en\|volume=33\|issue=1\|doi=10.1186/1756-9966-33-33\|issn=1756-9966}}</ref><ref name=":2">{{Cite journal\|last=Demicco\|first=Elizabeth G.\|last2=Wani\|first2=Khalida\|last3=Ingram\|first3=Davis\|last4=Wagner\|first4=Michael\|last5=Maki\|first5=Robert G.\|last6=Rizzo\|first6=Anthony\|last7=Meeker\|first7=Alan\|last8=Lazar\|first8=Alexander J.\|last9=Wang\|first9=Wei-Lien\|date=2018-11\|title=TERT promoter mutations in solitary fibrous tumour\|url=https://pubmed.ncbi.nlm.nih.gov/29985536\|journal=Histopathology\|volume=73\|issue=5\|pages=843–851\|doi=10.1111/his.13703\|issn=1365-2559\|pmid=29985536}}</ref><ref name=":6">{{Cite journal\|last=Yao\|first=Chen-chen\|last2=Zhou\|first2=Jian\|last3=Li\|first3=Xiao\|last4=Yang\|first4=Jun\|last5=Chen\|first5=Gang\|last6=Wei\|first6=Jia\|last7=Fan\|first7=Qin-he\|last8=Gong\|first8=Qi-xing\|date=2024-01-04\|title=Prognostic analysis of extrameningeal solitary fibrous tumor using the modified Demicco model: a clinicopathologic study of 111 Chinese cases\|url=https://www.frontiersin.org/articles/10.3389/fonc.2023.1272090/full\|journal=Frontiers in Oncology\|volume=13\|doi=10.3389/fonc.2023.1272090\|issn=2234-943X}}</ref><ref>{{Cite journal\|last=Machado\|first=Isidro\|last2=Morales\|first2=Gema Nieto\|last3=Cruz\|first3=Julia\|last4=Lavernia\|first4=Javier\|last5=Giner\|first5=Francisco\|last6=Navarro\|first6=Samuel\|last7=Ferrandez\|first7=Antonio\|last8=Llombart-Bosch\|first8=Antonio\|date=2020-04\|title=Solitary fibrous tumor: a case series identifying pathological adverse factors—implications for risk stratification and classification\|url=http://link.springer.com/10.1007/s00428-019-02660-3\|journal=Virchows Archiv\|language=en\|volume=476\|issue=4\|pages=597–607\|doi=10.1007/s00428-019-02660-3\|issn=0945-6317}}</ref>		\|''TERT'' promoter mutations were more frequent in tumors with higher risk of metastasis, have a significant association with malignant SFTs, and may identify intermediate-risk tumors with poorer prognosis.<ref name=":0" /><ref name=":1" /><ref name=":3">{{Cite journal\|last=Bahrami\|first=Armita\|last2=Lee\|first2=Seungjae\|last3=Schaefer\|first3=Inga-Marie\|last4=Boland\|first4=Jennifer M\|last5=Patton\|first5=Kurt T\|last6=Pounds\|first6=Stanley\|last7=Fletcher\|first7=Christopher D\|date=2016-12\|title=TERT promoter mutations and prognosis in solitary fibrous tumor\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222023328\|journal=Modern Pathology\|language=en\|volume=29\|issue=12\|pages=1511–1522\|doi=10.1038/modpathol.2016.126}}</ref><ref>{{Cite journal\|last=Killela\|first=Patrick J.\|last2=Reitman\|first2=Zachary J.\|last3=Jiao\|first3=Yuchen\|last4=Bettegowda\|first4=Chetan\|last5=Agrawal\|first5=Nishant\|last6=Diaz\|first6=Luis A.\|last7=Friedman\|first7=Allan H.\|last8=Friedman\|first8=Henry\|last9=Gallia\|first9=Gary L.\|date=2013-04-09\|title=TERT promoter mutations occur frequently in gliomas and a subset of tumors derived from cells with low rates of self-renewal\|url=https://pnas.org/doi/full/10.1073/pnas.1303607110\|journal=Proceedings of the National Academy of Sciences\|language=en\|volume=110\|issue=15\|pages=6021–6026\|doi=10.1073/pnas.1303607110\|issn=0027-8424\|pmc=PMC3625331\|pmid=23530248}}</ref><ref>{{Cite journal\|last=Koelsche\|first=Christian\|last2=Renner\|first2=Marcus\|last3=Hartmann\|first3=Wolfgang\|last4=Brandt\|first4=Regine\|last5=Lehner\|first5=Burkhard\|last6=Waldburger\|first6=Nina\|last7=Alldinger\|first7=Ingo\|last8=Schmitt\|first8=Thomas\|last9=Egerer\|first9=Gerlinde\|date=2014-12\|title=TERT promoter hotspot mutations are recurrent in myxoid liposarcomas but rare in other soft tissue sarcoma entities\|url=https://jeccr.biomedcentral.com/articles/10.1186/1756-9966-33-33\|journal=Journal of Experimental & Clinical Cancer Research\|language=en\|volume=33\|issue=1\|doi=10.1186/1756-9966-33-33\|issn=1756-9966}}</ref><ref name=":2">{{Cite journal\|last=Demicco\|first=Elizabeth G.\|last2=Wani\|first2=Khalida\|last3=Ingram\|first3=Davis\|last4=Wagner\|first4=Michael\|last5=Maki\|first5=Robert G.\|last6=Rizzo\|first6=Anthony\|last7=Meeker\|first7=Alan\|last8=Lazar\|first8=Alexander J.\|last9=Wang\|first9=Wei-Lien\|date=2018-11\|title=TERT promoter mutations in solitary fibrous tumour\|url=https://pubmed.ncbi.nlm.nih.gov/29985536\|journal=Histopathology\|volume=73\|issue=5\|pages=843–851\|doi=10.1111/his.13703\|issn=1365-2559\|pmid=29985536}}</ref><ref name=":6">{{Cite journal\|last=Yao\|first=Chen-chen\|last2=Zhou\|first2=Jian\|last3=Li\|first3=Xiao\|last4=Yang\|first4=Jun\|last5=Chen\|first5=Gang\|last6=Wei\|first6=Jia\|last7=Fan\|first7=Qin-he\|last8=Gong\|first8=Qi-xing\|date=2024-01-04\|title=Prognostic analysis of extrameningeal solitary fibrous tumor using the modified Demicco model: a clinicopathologic study of 111 Chinese cases\|url=https://www.frontiersin.org/articles/10.3389/fonc.2023.1272090/full\|journal=Frontiers in Oncology\|volume=13\|doi=10.3389/fonc.2023.1272090\|issn=2234-943X}}</ref><ref>{{Cite journal\|last=Machado\|first=Isidro\|last2=Morales\|first2=Gema Nieto\|last3=Cruz\|first3=Julia\|last4=Lavernia\|first4=Javier\|last5=Giner\|first5=Francisco\|last6=Navarro\|first6=Samuel\|last7=Ferrandez\|first7=Antonio\|last8=Llombart-Bosch\|first8=Antonio\|date=2020-04\|title=Solitary fibrous tumor: a case series identifying pathological adverse factors—implications for risk stratification and classification\|url=http://link.springer.com/10.1007/s00428-019-02660-3\|journal=Virchows Archiv\|language=en\|volume=476\|issue=4\|pages=597–607\|doi=10.1007/s00428-019-02660-3\|issn=0945-6317}}</ref>
	\|-		\|-
	\|''TP53''		\|''TP53''
Line 118:		Line 118:
	\|P		\|P
	\|No		\|No
	\|Mutations of TP53 have been associated with malignant and dedifferentiated SFTs.<ref name=":0" /><ref name=":6" /><ref>{{Cite journal\|last=Dagrada\|first=Gian P\|last2=Spagnuolo\|first2=Rosalin D\|last3=Mauro\|first3=Valentina\|last4=Tamborini\|first4=Elena\|last5=Cesana\|first5=Luca\|last6=Gronchi\|first6=Alessandro\|last7=Stacchiotti\|first7=Silvia\|last8=Pierotti\|first8=Marco A\|last9=Negri\|first9=Tiziana\|date=2015-08\|title=Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222013904\|journal=Modern Pathology\|language=en\|volume=28\|issue=8\|pages=1074–1083\|doi=10.1038/modpathol.2015.70}}</ref><ref>{{Cite journal\|last=Kurisaki-Arakawa\|first=Aiko\|last2=Akaike\|first2=Keisuke\|last3=Hara\|first3=Kieko\|last4=Arakawa\|first4=Atsushi\|last5=Takahashi\|first5=Michiko\|last6=Mitani\|first6=Keiko\|last7=Yao\|first7=Takashi\|last8=Saito\|first8=Tsuyoshi\|date=2014-11\|title=A case of dedifferentiated solitary fibrous tumor in the pelvis with TP53 mutation\|url=https://pubmed.ncbi.nlm.nih.gov/25015562\|journal=Virchows Archiv: An International Journal of Pathology\|volume=465\|issue=5\|pages=615–621\|doi=10.1007/s00428-014-1625-3\|issn=1432-2307\|pmid=25015562}}</ref><ref>{{Cite journal\|last=Nonaka\|first=Haruna\|last2=Kandori\|first2=Shuya\|last3=Nitta\|first3=Satoshi\|last4=Shiga\|first4=Masanobu\|last5=Nagumo\|first5=Yoshiyuki\|last6=Kimura\|first6=Tomokazu\|last7=Kawahara\|first7=Takashi\|last8=Negoro\|first8=Hiromitsu\|last9=Hoshi\|first9=Akio\|date=2021\|title=Case Report: Molecular Characterization of Aggressive Malignant Retroperitoneal Solitary Fibrous Tumor: A Case Study\|url=https://pubmed.ncbi.nlm.nih.gov/35004271\|journal=Frontiers in Oncology\|volume=11\|pages=736969\|doi=10.3389/fonc.2021.736969\|issn=2234-943X\|pmc=8727594\|pmid=35004271}}</ref>		\|Mutations of ''TP53'' have been associated with malignant and dedifferentiated SFTs.<ref name=":0" /><ref name=":6" /><ref>{{Cite journal\|last=Dagrada\|first=Gian P\|last2=Spagnuolo\|first2=Rosalin D\|last3=Mauro\|first3=Valentina\|last4=Tamborini\|first4=Elena\|last5=Cesana\|first5=Luca\|last6=Gronchi\|first6=Alessandro\|last7=Stacchiotti\|first7=Silvia\|last8=Pierotti\|first8=Marco A\|last9=Negri\|first9=Tiziana\|date=2015-08\|title=Solitary fibrous tumors: loss of chimeric protein expression and genomic instability mark dedifferentiation\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222013904\|journal=Modern Pathology\|language=en\|volume=28\|issue=8\|pages=1074–1083\|doi=10.1038/modpathol.2015.70}}</ref><ref>{{Cite journal\|last=Kurisaki-Arakawa\|first=Aiko\|last2=Akaike\|first2=Keisuke\|last3=Hara\|first3=Kieko\|last4=Arakawa\|first4=Atsushi\|last5=Takahashi\|first5=Michiko\|last6=Mitani\|first6=Keiko\|last7=Yao\|first7=Takashi\|last8=Saito\|first8=Tsuyoshi\|date=2014-11\|title=A case of dedifferentiated solitary fibrous tumor in the pelvis with TP53 mutation\|url=https://pubmed.ncbi.nlm.nih.gov/25015562\|journal=Virchows Archiv: An International Journal of Pathology\|volume=465\|issue=5\|pages=615–621\|doi=10.1007/s00428-014-1625-3\|issn=1432-2307\|pmid=25015562}}</ref><ref>{{Cite journal\|last=Nonaka\|first=Haruna\|last2=Kandori\|first2=Shuya\|last3=Nitta\|first3=Satoshi\|last4=Shiga\|first4=Masanobu\|last5=Nagumo\|first5=Yoshiyuki\|last6=Kimura\|first6=Tomokazu\|last7=Kawahara\|first7=Takashi\|last8=Negoro\|first8=Hiromitsu\|last9=Hoshi\|first9=Akio\|date=2021\|title=Case Report: Molecular Characterization of Aggressive Malignant Retroperitoneal Solitary Fibrous Tumor: A Case Study\|url=https://pubmed.ncbi.nlm.nih.gov/35004271\|journal=Frontiers in Oncology\|volume=11\|pages=736969\|doi=10.3389/fonc.2021.736969\|issn=2234-943X\|pmc=8727594\|pmid=35004271}}</ref>
	\|-		\|-
	\|''APAF1''		\|''APAF1''
Line 126:		Line 126:
	\|P		\|P
	\|No		\|No
	\|Alteration of APAF1 results in gain of a stop codon. The gene is inactivated by DNA methylation of the promoter region. Decreased APAF1 is considered to lead to inhibition of apoptosis. This alteration and decreased APAF1 mRNA expression was observed in metastatic SFT.<ref name=":1" />		\|Alteration of ''APAF1'' results in gain of a stop codon. The gene is inactivated by DNA methylation of the promoter region. Decreased ''APAF1'' is considered to lead to inhibition of apoptosis. This alteration and decreased ''APAF1'' mRNA expression was observed in metastatic SFT.<ref name=":1" />
	\|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.		\|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.

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	!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome		!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
	\|-		\|-
	\|NAB2::STAT6; Activating mutation		\|''NAB2::STAT6''; Activating mutation
	\|EGR Pathway		\|EGR Pathway
	\|Increased activation of EGR1		\|Increased activation of EGR1
Line 145:		Line 145:
	# '''Fusion testing'''		# '''Fusion testing'''
	#* Next generation sequencing (NGS) using mRNA targeting the fusion		#* Next generation sequencing (NGS) using mRNA targeting the fusion
	#* Dual color dual fusion fluorescence in situ hybridization (FISH) probe targeting both genes<ref>{{Cite journal\|last=Kouba\|first=Erik\|last2=Simper\|first2=Novae B.\|last3=Chen\|first3=Shaoxiong\|last4=Williamson\|first4=Sean R.\|last5=Grignon\|first5=David J.\|last6=Eble\|first6=John N.\|last7=MacLennan\|first7=Gregory T.\|last8=Montironi\|first8=Rodolfo\|last9=Lopez-Beltran\|first9=Antonio\|date=2017-06-01\|title=Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene\|url=https://jcp.bmj.com/content/70/6/508\|journal=Journal of Clinical Pathology\|language=en\|volume=70\|issue=6\|pages=508–514\|doi=10.1136/jclinpath-2016-204088\|issn=0021-9746\|pmid=27802414}}</ref>		#* Dual color dual fusion fluorescence ''in situ'' hybridization (FISH) probe targeting both genes<ref>{{Cite journal\|last=Kouba\|first=Erik\|last2=Simper\|first2=Novae B.\|last3=Chen\|first3=Shaoxiong\|last4=Williamson\|first4=Sean R.\|last5=Grignon\|first5=David J.\|last6=Eble\|first6=John N.\|last7=MacLennan\|first7=Gregory T.\|last8=Montironi\|first8=Rodolfo\|last9=Lopez-Beltran\|first9=Antonio\|date=2017-06-01\|title=Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene\|url=https://jcp.bmj.com/content/70/6/508\|journal=Journal of Clinical Pathology\|language=en\|volume=70\|issue=6\|pages=508–514\|doi=10.1136/jclinpath-2016-204088\|issn=0021-9746\|pmid=27802414}}</ref>
	#** a direct confirmation for ''NAB2::STAT6'' fusion		#** a direct confirmation for ''NAB2::STAT6'' fusion
	# '''Breakpoint detection'''		# '''Breakpoint detection'''
	#* Breakpoint fluorescence in situ hybridization (FISH) probe for ''STAT6''		#* Breakpoint fluorescence ''in situ'' hybridization (FISH) probe for ''STAT6''

	==Familial Forms==		==Familial Forms==

Yiting.Li: spell check

2025-12-15T18:18:18Z

spell check

← Older revision		Revision as of 13:18, 15 December 2025
Line 113:		Line 113:
	\|''TP53''		\|''TP53''
	<br />		<br />
	\|Missense or ~~fram~~-shift mutation in exon 5 or exon 6 <ref name=":3" />		\|Missense or frame-shift mutation in exon 5 or exon 6 <ref name=":3" />
	\|Tumor Supressor Gene		\|Tumor Supressor Gene
	\|Common		\|Common

Yiting.Li: move target therapy to clinical relevance details/other notes of gene rearrangements. modify authorship section.

2025-12-15T14:44:45Z

move target therapy to clinical relevance details/other notes of gene rearrangements. modify authorship section.

← Older revision		Revision as of 09:44, 15 December 2025
Line 5:		Line 5:
	{{Under Construction}}		{{Under Construction}}
	==Primary Author(s)*==		==Primary Author(s)*==
	Yiting Li*, Reba Daniel and Shashi Shetty		Yiting Li, Reba Daniel and Shashi Shetty
	==WHO Classification of Disease==		==WHO Classification of Disease==

Line 54:		Line 54:
	\|D		\|D
	\|Y (WHO)		\|Y (WHO)
	\|''NAB2''::''STAT6'' gene fusions are pathognomonic for SFT. Many different breakpoints in the exons and introns are associated with this fusion. The most common fusion variants: ''NAB2''ex4-''STAT6''ex2; ''NAB2''ex6-''STAT6''ex16/17. <ref name=":4" /><ref name=":5" />		\|''NAB2''::''STAT6'' gene fusions are pathognomonic for SFT. Many different breakpoints in the exons and introns are associated with this fusion. The most common fusion variants: ''NAB2''ex4-''STAT6''ex2; ''NAB2''ex6-''STAT6''ex16/17. <ref name=":4" /><ref name=":5" /> Antiangiogenic therapy showed activity in advanced and progressive SFT<ref>{{Cite journal\|last=de Bernardi\|first=Axel\|last2=Dufresne\|first2=Armelle\|last3=Mishellany\|first3=Florence\|last4=Blay\|first4=Jean-Yves\|last5=Ray-Coquard\|first5=Isabelle\|last6=Brahmi\|first6=Mehdi\|date=2022-02-20\|title=Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond\|url=https://www.mdpi.com/2072-6694/14/4/1064\|journal=Cancers\|language=en\|volume=14\|issue=4\|pages=1064\|doi=10.3390/cancers14041064\|issn=2072-6694}}</ref>
	\|}		\|}

Line 158:		Line 158:
	** nuclear staining		** nuclear staining
	** high sensitivity and specificity<ref>{{Cite journal\|last=Doyle\|first=Leona A\|last2=Vivero\|first2=Marina\|last3=Fletcher\|first3=Christopher DM\|last4=Mertens\|first4=Fredrik\|last5=Hornick\|first5=Jason L\|date=2014-03\|title=Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222018294\|journal=Modern Pathology\|language=en\|volume=27\|issue=3\|pages=390–395\|doi=10.1038/modpathol.2013.164}}</ref>		** high sensitivity and specificity<ref>{{Cite journal\|last=Doyle\|first=Leona A\|last2=Vivero\|first2=Marina\|last3=Fletcher\|first3=Christopher DM\|last4=Mertens\|first4=Fredrik\|last5=Hornick\|first5=Jason L\|date=2014-03\|title=Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222018294\|journal=Modern Pathology\|language=en\|volume=27\|issue=3\|pages=390–395\|doi=10.1038/modpathol.2013.164}}</ref>
	~~'''Target therapy'''~~
	* Antiangiogenic therapy showed activity in advanced and progressive SFT<ref>{{Cite journal\|last=de Bernardi\|first=Axel\|last2=Dufresne\|first2=Armelle\|last3=Mishellany\|first3=Florence\|last4=Blay\|first4=Jean-Yves\|last5=Ray-Coquard\|first5=Isabelle\|last6=Brahmi\|first6=Mehdi\|date=2022-02-20\|title=Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond\|url=https://www.mdpi.com/2072-6694/14/4/1064\|journal=Cancers\|language=en\|volume=14\|issue=4\|pages=1064\|doi=10.3390/cancers14041064\|issn=2072-6694}}</ref>
	==Links==		==Links==
	None		None

Yiting.Li: add target therapy

2025-12-14T01:30:34Z

add target therapy

← Older revision		Revision as of 20:30, 13 December 2025
Line 5:		Line 5:
	{{Under Construction}}		{{Under Construction}}
	==Primary Author(s)*==		==Primary Author(s)*==
	Reba Daniel and Shashi Shetty		Yiting Li*, Reba Daniel and Shashi Shetty
	==WHO Classification of Disease==		==WHO Classification of Disease==

Line 35:		Line 35:
	\|-		\|-
	\|Not Recommended		\|Not Recommended
	\|~~Solitary~~ fibrous tumour ~~/ Haemangiopericytoma; Haemangiopericytoma~~		\|Haemangiopericytoma; Giant cell angiofibroma; Benign solitary fibrous tumour
	\|}		\|}

Line 145:		Line 145:
	# '''Fusion testing'''		# '''Fusion testing'''
	#* Next generation sequencing (NGS) using mRNA targeting the fusion		#* Next generation sequencing (NGS) using mRNA targeting the fusion
	#* Dual color dual fusion probe targeting both genes<ref>{{Cite journal\|last=Kouba\|first=Erik\|last2=Simper\|first2=Novae B.\|last3=Chen\|first3=Shaoxiong\|last4=Williamson\|first4=Sean R.\|last5=Grignon\|first5=David J.\|last6=Eble\|first6=John N.\|last7=MacLennan\|first7=Gregory T.\|last8=Montironi\|first8=Rodolfo\|last9=Lopez-Beltran\|first9=Antonio\|date=2017-06-01\|title=Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene\|url=https://jcp.bmj.com/content/70/6/508\|journal=Journal of Clinical Pathology\|language=en\|volume=70\|issue=6\|pages=508–514\|doi=10.1136/jclinpath-2016-204088\|issn=0021-9746\|pmid=27802414}}</ref>		#* Dual color dual fusion fluorescence in situ hybridization (FISH) probe targeting both genes<ref>{{Cite journal\|last=Kouba\|first=Erik\|last2=Simper\|first2=Novae B.\|last3=Chen\|first3=Shaoxiong\|last4=Williamson\|first4=Sean R.\|last5=Grignon\|first5=David J.\|last6=Eble\|first6=John N.\|last7=MacLennan\|first7=Gregory T.\|last8=Montironi\|first8=Rodolfo\|last9=Lopez-Beltran\|first9=Antonio\|date=2017-06-01\|title=Solitary fibrous tumour of the genitourinary tract: a clinicopathological study of 11 cases and their association with the NAB2-STAT6 fusion gene\|url=https://jcp.bmj.com/content/70/6/508\|journal=Journal of Clinical Pathology\|language=en\|volume=70\|issue=6\|pages=508–514\|doi=10.1136/jclinpath-2016-204088\|issn=0021-9746\|pmid=27802414}}</ref>
	#** a direct confirmation for ''NAB2::STAT6'' fusion		#** a direct confirmation for ''NAB2::STAT6'' fusion
	# '''Breakpoint detection'''		# '''Breakpoint detection'''
	#* Breakpoint fluorescence in situ hybridization (FISH) probe for ''STAT6''		#* Breakpoint fluorescence in situ hybridization (FISH) probe for ''STAT6''
	~~# '''Immunohistochemistry testing'''~~
	~~#* Immunohistochemistry testing, STAT6 (signal transducer and activator of transcription 6) immunostain~~
	~~#** nuclear staining~~
	~~#** high sensitivity and specificity~~

	==Familial Forms==		==Familial Forms==
Line 158:		Line 154:
	==Additional Information==		==Additional Information==

	~~None~~		'''Immunohistochemistry testing'''
			* STAT6 (signal transducer and activator of transcription 6) immunostain
			** nuclear staining
			** high sensitivity and specificity<ref>{{Cite journal\|last=Doyle\|first=Leona A\|last2=Vivero\|first2=Marina\|last3=Fletcher\|first3=Christopher DM\|last4=Mertens\|first4=Fredrik\|last5=Hornick\|first5=Jason L\|date=2014-03\|title=Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics\|url=https://linkinghub.elsevier.com/retrieve/pii/S0893395222018294\|journal=Modern Pathology\|language=en\|volume=27\|issue=3\|pages=390–395\|doi=10.1038/modpathol.2013.164}}</ref>
			'''Target therapy'''
			* Antiangiogenic therapy showed activity in advanced and progressive SFT<ref>{{Cite journal\|last=de Bernardi\|first=Axel\|last2=Dufresne\|first2=Armelle\|last3=Mishellany\|first3=Florence\|last4=Blay\|first4=Jean-Yves\|last5=Ray-Coquard\|first5=Isabelle\|last6=Brahmi\|first6=Mehdi\|date=2022-02-20\|title=Novel Therapeutic Options for Solitary Fibrous Tumor: Antiangiogenic Therapy and Beyond\|url=https://www.mdpi.com/2072-6694/14/4/1064\|journal=Cancers\|language=en\|volume=14\|issue=4\|pages=1064\|doi=10.3390/cancers14041064\|issn=2072-6694}}</ref>
	==Links==		==Links==
	None		None

Yiting.Li: modify the gene mutation part, check the references and corresponding sentence.

2025-11-23T21:06:09Z

modify the gene mutation part, check the references and corresponding sentence.

Show changes

Yiting.Li: merge previous version to the new template, only keep genetics related context

2025-11-23T17:04:57Z

merge previous version to the new template, only keep genetics related context

Show changes