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Update NAB2-STAT6 fusions as specific marker for SFT/hemangiopericytoma
Jensflorian (talk | contribs) (Update NAB2-STAT6 fusions as specific marker for SFT/hemangiopericytoma) |
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==General== | ==General== | ||
*Grouped with ''hemangiopericytoma'' in the WHO classification - as it is thought to be the same tumour.<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref> | *Grouped with ''hemangiopericytoma'' in the WHO classification - as it is thought to be the same tumour because both share the same molecular alteration.<ref name=Ref_WMSP609>{{Ref WMSP|609}}</ref><ref>{{Cite journal | last1 = Schweizer | first1 = L. | last2 = Koelsche | first2 = C. | last3 = Sahm | first3 = F. | last4 = Piro | first4 = RM. | last5 = Capper | first5 = D. | last6 = Reuss | first6 = DE. | last7 = Pusch | first7 = S. | last8 = Habel | first8 = A. | last9 = Meyer | first9 = J. | title = Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein. | journal = Acta Neuropathol | volume = 125 | issue = 5 | pages = 651-8 | month = May | year = 2013 | doi = 10.1007/s00401-013-1117-6 | PMID = 23575898 }}</ref> | ||
*May be benign ''or'' malignant; more commonly benign.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9]. Accessed on: 25 June 2010.</ref><ref>URL: [http://wjso.com/content/6/1/86 http://wjso.com/content/6/1/86]. Accessed on: 25 June 2010.</ref> | *May be benign ''or'' malignant; more commonly benign.<ref>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9]. Accessed on: 25 June 2010.</ref><ref>URL: [http://wjso.com/content/6/1/86 http://wjso.com/content/6/1/86]. Accessed on: 25 June 2010.</ref> | ||
*May be associated with hypoglycemia. | *May be associated with hypoglycemia. | ||
**Known as ''Doege-Potter syndrome''.<ref name=pmid1474302>{{Cite journal | last1 = Roy | first1 = TM. | last2 = Burns | first2 = MV. | last3 = Overly | first3 = DJ. | last4 = Curd | first4 = BT. | title = Solitary fibrous tumor of the pleura with hypoglycemia: the Doege-Potter syndrome. | journal = J Ky Med Assoc | volume = 90 | issue = 11 | pages = 557-60 | month = Nov | year = 1992 | doi = | PMID = 1474302 }}</ref> | **Known as ''Doege-Potter syndrome''.<ref name=pmid1474302>{{Cite journal | last1 = Roy | first1 = TM. | last2 = Burns | first2 = MV. | last3 = Overly | first3 = DJ. | last4 = Curd | first4 = BT. | title = Solitary fibrous tumor of the pleura with hypoglycemia: the Doege-Potter syndrome. | journal = J Ky Med Assoc | volume = 90 | issue = 11 | pages = 557-60 | month = Nov | year = 1992 | doi = | PMID = 1474302 }}</ref> | ||
*Leptomeningeal SFTs/hemangiopericytomas are classified as WHO grade II, anaplastic forms as WHO grade III. | |||
==Gross== | ==Gross== | ||
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*CD99 ~70% +ve. | *CD99 ~70% +ve. | ||
*BCL2 ~50% +ve. | *BCL2 ~50% +ve. | ||
*Stat6 nuclear +ve.<ref>{{Cite journal | last1 = Cheah | first1 = AL. | last2 = Billings | first2 = SD. | last3 = Goldblum | first3 = JR. | last4 = Carver | first4 = P. | last5 = Tanas | first5 = MZ. | last6 = Rubin | first6 = BP. | title = STAT6 rabbit monoclonal antibody is a robust diagnostic tool for the distinction of solitary fibrous tumour from its mimics. | journal = Pathology | volume = 46 | issue = 5 | pages = 389-95 | month = Aug | year = 2014 | doi = 10.1097/PAT.0000000000000122 | PMID = 24977739 }}</ref> | |||
==Molecular== | |||
*NAB2/STAT6 fusions.<ref>{{Cite journal | last1 = Mohajeri | first1 = A. | last2 = Tayebwa | first2 = J. | last3 = Collin | first3 = A. | last4 = Nilsson | first4 = J. | last5 = Magnusson | first5 = L. | last6 = von Steyern | first6 = FV. | last7 = Brosjö | first7 = O. | last8 = Domanski | first8 = HA. | last9 = Larsson | first9 = O. | title = Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor. | journal = Genes Chromosomes Cancer | volume = 52 | issue = 10 | pages = 873-86 | month = Oct | year = 2013 | doi = 10.1002/gcc.22083 | PMID = 23761323 }}</ref><ref>{{Cite journal | last1 = Robinson | first1 = DR. | last2 = Wu | first2 = YM. | last3 = Kalyana-Sundaram | first3 = S. | last4 = Cao | first4 = X. | last5 = Lonigro | first5 = RJ. | last6 = Sung | first6 = YS. | last7 = Chen | first7 = CL. | last8 = Zhang | first8 = L. | last9 = Wang | first9 = R. | title = Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing. | journal = Nat Genet | volume = 45 | issue = 2 | pages = 180-5 | month = Feb | year = 2013 | doi = 10.1038/ng.2509 | PMID = 23313952 }}</ref> | |||
==DDx== | |||
*[[Meningioma]] | |||
*Cellular [[angiofibroma]] | |||
*[[Myofibroblastoma]] | |||
*Benign [[fibrous histiocytoma]] | |||
*[[Dermatofibrosarcoma protruberans]] | |||
*[[Fibromyxoid sarcoma]] | |||
==See also== | ==See also== | ||
*[[Fibroblastic/myofibroblastic tumours]]. | *[[Fibroblastic/myofibroblastic tumours]]. | ||
*[[Hemangiopericytoma]] | |||
==References== | ==References== | ||
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[[Category:Fibroblastic/myofibroblastic tumours]] | [[Category:Fibroblastic/myofibroblastic tumours]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology]] | |||