Difference between revisions of "Solitary fibrous tumour"
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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== | ||
| Line 78: | Line 93: | ||
[[Category:Fibroblastic/myofibroblastic tumours]] | [[Category:Fibroblastic/myofibroblastic tumours]] | ||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Neuropathology]] | |||
Revision as of 08:19, 20 May 2015
| Solitary fibrous tumour | |
|---|---|
| Diagnosis in short | |
 Solitary fibrous tumour. H&E stain. | |
|
| |
| LM | spindle cells in a patternless pattern, hemangiopericytoma-like areas (staghorn vessels), keloid-like collagen bundles, +/-well-circumscribed (common) |
| Subtypes | benign (common), malignant (uncommon) |
| IHC | CD34 ~90% +ve, CD99 ~70% +ve, BCL2 ~50% +ve |
| Site | soft tissue - fibroblastic/myofibroblastic tumours, pleura |
|
| |
| Syndromes | Doege-Potter syndrome |
|
| |
| Prognosis | usu. good |
Solitary fibrous tumour, abbreviated SFT, is a type of soft tissue tumour that fits in the fibroblastic/myofibroblastic tumours. It is usually benign.
SFT of the pleura is dealt with in a separate article solitary fibrous tumour of the pleura.
General
- Grouped with hemangiopericytoma in the WHO classification - as it is thought to be the same tumour because both share the same molecular alteration.[1][2]
- May be benign or malignant; more commonly benign.[3][4]
- May be associated with hypoglycemia.
- Known as Doege-Potter syndrome.[5]
- Leptomeningeal SFTs/hemangiopericytomas are classified as WHO grade II, anaplastic forms as WHO grade III.
Gross
- Soft tissue mass.
Microscopic
Features - benign:
- Spindle cells in a patternless pattern.
- Occasionally epithelioid cells - rare.[6]
- Hemangiopericytoma-like area (staghorn vessels).
- Keloid-like collagen bundles - key feature.
- +/-Well-circumscribed (common).
Criteria for malignancy:[1]
- Necrosis.
- Mitoses >4/10 HPF -- definition suffers from HPFitis.
- Increased cellularity.
- Marked nuclear atypia.
- Infiltrative margin.
Images
Benign SFT - low mag. (WC)
Benign SFT - intermed. mag. (WC)
Benign SFT - high mag. (WC)
www:
IHC
- CD34 ~90% +ve.
- CD99 ~70% +ve.
- BCL2 ~50% +ve.
- Stat6 nuclear +ve.[7]
Molecular
DDx
- Meningioma
- Cellular angiofibroma
- Myofibroblastoma
- Benign fibrous histiocytoma
- Dermatofibrosarcoma protruberans
- Fibromyxoid sarcoma
See also
References
- ↑ 1.0 1.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 609. ISBN 978-0781765275.
- ↑ Schweizer, L.; Koelsche, C.; Sahm, F.; Piro, RM.; Capper, D.; Reuss, DE.; Pusch, S.; Habel, A. et al. (May 2013). "Meningeal hemangiopericytoma and solitary fibrous tumors carry the NAB2-STAT6 fusion and can be diagnosed by nuclear expression of STAT6 protein.". Acta Neuropathol 125 (5): 651-8. doi:10.1007/s00401-013-1117-6. PMID 23575898.
- ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970528-9. Accessed on: 25 June 2010.
- ↑ URL: http://wjso.com/content/6/1/86. Accessed on: 25 June 2010.
- ↑ Roy, TM.; Burns, MV.; Overly, DJ.; Curd, BT. (Nov 1992). "Solitary fibrous tumor of the pleura with hypoglycemia: the Doege-Potter syndrome.". J Ky Med Assoc 90 (11): 557-60. PMID 1474302.
- ↑ Martorell, M.; Pérez-Vallés, A.; Gozalbo, F.; Garcia-Garcia, JA.; Gutierrez, J.; Gaona, J. (2007). "Solitary fibrous tumor of the thigh with epithelioid features: a case report.". Diagn Pathol 2: 19. doi:10.1186/1746-1596-2-19. PMID 17577399.
- ↑ Cheah, AL.; Billings, SD.; Goldblum, JR.; Carver, P.; Tanas, MZ.; Rubin, BP. (Aug 2014). "STAT6 rabbit monoclonal antibody is a robust diagnostic tool for the distinction of solitary fibrous tumour from its mimics.". Pathology 46 (5): 389-95. doi:10.1097/PAT.0000000000000122. PMID 24977739.
- ↑ Mohajeri, A.; Tayebwa, J.; Collin, A.; Nilsson, J.; Magnusson, L.; von Steyern, FV.; Brosjö, O.; Domanski, HA. et al. (Oct 2013). "Comprehensive genetic analysis identifies a pathognomonic NAB2/STAT6 fusion gene, nonrandom secondary genomic imbalances, and a characteristic gene expression profile in solitary fibrous tumor.". Genes Chromosomes Cancer 52 (10): 873-86. doi:10.1002/gcc.22083. PMID 23761323.
- ↑ Robinson, DR.; Wu, YM.; Kalyana-Sundaram, S.; Cao, X.; Lonigro, RJ.; Sung, YS.; Chen, CL.; Zhang, L. et al. (Feb 2013). "Identification of recurrent NAB2-STAT6 gene fusions in solitary fibrous tumor by integrative sequencing.". Nat Genet 45 (2): 180-5. doi:10.1038/ng.2509. PMID 23313952.