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Synovial sarcoma (view source)

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#redirect [[Soft_tissue_lesions#Synovial sarcoma]]
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Monophasic_synovial_sarcoma_-_high_mag.jpg
| Width      =
| Caption    = Monophasic synovial sarcoma. [[H&E stain]].
| Micro      = one of the following (1) spindle cell sarcoma with features of hemangiopericytoma, i.e. [[staghorn vessels]]; (2) biphasic synovial sarcoma (spindle cells with features of hemangiopericytoma, epitheliod glands or nests); (3) primitive round cell type
| Subtypes  =
| LMDDx      = [[MPNST]], [[fibrosarcoma]], [[small round cell tumours]]
| Stains    =
| IHC        = Vimentin +ve, EMA +ve, BCL2 +ve, CD99 +ve.
| EM        =
| Molecular  = t(X;18)
| IF        =
| Gross      = usually lower extremity, usually close to a joint
| Grossing  =
| Site      = [[soft tissue lesions|soft tissue]]
| Assdx      =
| Syndromes  =
| Clinicalhx = young adults or adolescents
| Signs      = mass +/-pain
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = poor
| Other      =
| ClinDDx    =
}}
'''Synovial sarcoma''', abbreviated '''SS''', is an uncommon malignant soft tissue tumour, typically seen in young adults.
 
==General==
*Does not arise from cartilage.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Young adults or adolescents.
**Classic age: 30s.
*Poor prognosis.
 
Clinical:<ref name=pmid16973781>{{Cite journal  | last1 = Murphey | first1 = MD. | last2 = Gibson | first2 = MS. | last3 = Jennings | first3 = BT. | last4 = Crespo-Rodríguez | first4 = AM. | last5 = Fanburg-Smith | first5 = J. | last6 = Gajewski | first6 = DA. | title = From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation. | journal = Radiographics | volume = 26 | issue = 5 | pages = 1543-65 | month =  | year =  | doi = 10.1148/rg.265065084 | PMID = 16973781 | URL = http://radiographics.rsna.org/content/26/5/1543.long }}</ref>
*Present with soft palpable mass - slow growing - often for years.
*May present with pain.
**Uncommon finding in sarcomas.
 
==Gross==
Location:
*Usually close to a joint.
*Usually distal extremity ~65% of cases.<ref name=pmid16973781/>
**Upper extremity ~20% of cases.<ref name=pmid16973781/>
 
Appearance - often non-specific:
*Solid often lobulated +/- cystic component.
*Grey-yellow.
*Pushing border to ill-defined border.
 
Images:
*[http://www.sarcomaimages.com/images/db/synovial-sarcoma/synovial-sarcoma-01-gross-image,-sarcoma-images.jpg SS (sarcomaimages.com)].<ref>URL: [http://www.sarcomaimages.com/index.php?v=Synovial-Sarcoma http://www.sarcomaimages.com/index.php?v=Synovial-Sarcoma]. Accessed on: 2 April 2012.</ref>
*[http://www.tumorlibrary.com/case/detail.jsp?image_id=2462 SS (tumorlibrary.com)].
 
===Microscopic===
Comes in three (histologic) flavours:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref><ref>{{cite journal |author=Schaal CH, Navarro FC, Moraes Neto FA |title=Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma |journal=Int Braz J Urol |volume=30 |issue=3 |pages=210–3 |year=2004 |pmid=15689250 |doi= |url=http://www.brazjurol.com.br/may_june_2004/Schaal_ing_210_213.htm}}</ref>
#Spindle cell sarcoma with features of hemangiopericytoma, i.e. [[staghorn vessels]].
#Biphasic synovial sarcoma:
##Spindle cells with features of hemangiopericytoma.
##Epitheliod glands or nests.
#Primitive round cell type.
 
Features:
*[[Herring bone]] or [[vesicular pattern]] - '''key feature'''.
*Spindle cells.
*+/-Glandular component - typically more pink.
*+/-Calcification - uncommon.
**Extensive calcification = better prognosis.<ref name=pmid6282441>{{Cite journal  | last1 = Varela-Duran | first1 = J. | last2 = Enzinger | first2 = FM. | title = Calcifying synovial sarcoma. | journal = Cancer | volume = 50 | issue = 2 | pages = 345-52 | month = Jul | year = 1982 | doi =  | PMID = 6282441 }}</ref>
 
DDx:
*[[MPNST]].
**Can be difficult.
 
====Images====
<gallery>
Image:Monophasic_synovial_sarcoma_-_intermed_mag.jpg | Monophasic synovial sarcoma with staghorn vessels - intermed. mag. (WC/Nephron)
Image:Monophasic_synovial_sarcoma_-_high_mag.jpg | Monophasic synovial sarcoma with staghorn vessels - high mag. (WC/Nephron)
</gallery>
www:
*[http://www.scielo.br/img/revistas/ibju/v30n3/3a06f03.jpg Synovial sarcoma (scielo.br)].
*[http://www.humpath.com/spip.php?page=article&id_article=1965 Synovial sarcoma - collection of images (humpath.com)].
*[http://path.upmc.edu/cases/case292.html Synovial sarcoma - several images (upmc.edu)].
*[http://radiographics.rsna.org/content/26/5/1543/F14.expansion.html Biphasic SS (radiographics.rsna.org)].
*[http://radiographics.rsna.org/content/26/5/1543.long Monophasic SS (radiographics.rsna.org)].
 
===IHC===
Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
*Vimentin +ve.
*EMA +ve.
*BCL2 +ve.
*CD99 +ve.
 
Others:
*Beta-catenin +ve ~30-70%.<ref name=pmid16740029>{{cite journal |author=Horvai AE, Kramer MJ, O'Donnell R |title=Beta-catenin nuclear expression correlates with cyclin D1 expression in primary and metastatic synovial sarcoma: a tissue microarray study |journal=Arch. Pathol. Lab. Med. |volume=130 |issue=6 |pages=792–8 |year=2006 |month=June |pmid=16740029 |doi= |url=}}</ref>
*Cyclin D1 ~50%.<ref name=pmid16740029/><ref name=pmid15375433>{{cite journal |author=Ng TL, Gown AM, Barry TS, ''et al.'' |title=Nuclear beta-catenin in mesenchymal tumors |journal=Mod. Pathol. |volume=18 |issue=1 |pages=68–74 |year=2005 |month=January |pmid=15375433 |doi=10.1038/modpathol.3800272 |url=}}</ref>
*TLE1 +ve nuclear staining; not specific for synovial sarcoma.<ref name=pmid19363472>{{cite journal |author=Kosemehmetoglu K, Vrana JA, Folpe AL |title=TLE1 expression is not specific for synovial sarcoma: a whole section study of 163 soft tissue and bone neoplasms |journal=Mod. Pathol. |volume=22 |issue=7 |pages=872–8 |year=2009 |month=July |pmid=19363472 |doi=10.1038/modpathol.2009.47 |url=http://www.nature.com/modpathol/journal/v22/n7/full/modpathol200947a.html}}</ref><ref>{{cite journal |author=Seo SW, Lee H, Lee HI, Kim HS |title=The role of TLE1 in synovial sarcoma |journal=J Orthop Res |volume= |issue= |pages= |year=2011 |month=February |pmid=21319215 |doi=10.1002/jor.21318 |url=}}</ref>
 
Typically negative:<ref>URL: [http://path.upmc.edu/cases/case292/dx.html http://path.upmc.edu/cases/case292/dx.html]. Accessed on: 14 January 2012.</ref>
*CD34.
*S100 ~30% +ve.
*SMA.
 
Notes:
*Synovial sarcoma & MPNST:
**Both +ve: PGP9.5 (UCHL1<ref>{{OMIM|191342}}</ref>), S100, NGFR, CD56, CD99, vimentin.
**Synovial +ve: EMA, keratin, BCL2, TLE1.
**MPNST +ve: nestin, CD34.
 
Trivia:
*PGP in ''PGP9.5'' = protein gene product.<ref name=pmid6343558>{{Cite journal  | last1 = Doran | first1 = JF. | last2 = Jackson | first2 = P. | last3 = Kynoch | first3 = PA. | last4 = Thompson | first4 = RJ. | title = Isolation of PGP 9.5, a new human neurone-specific protein detected by high-resolution two-dimensional electrophoresis. | journal = J Neurochem | volume = 40 | issue = 6 | pages = 1542-7 | month = Jun | year = 1983 | doi =  | PMID = 6343558 }}</ref>
 
===EM===
Features:<ref name=pmid9930576>{{Cite journal  | last1 = Fisher | first1 = C. | title = Synovial sarcoma. | journal = Ann Diagn Pathol | volume = 2 | issue = 6 | pages = 401-21 | month = Dec | year = 1998 | doi =  | PMID = 9930576 }}</ref>
*Biphasic tumour have biphasic ultrastructural features (unlike spindle cell carcinoma and [[epithelioid sarcoma]]).
*Epithelioid component is adenocarcinoma-like - they have:
**Intermediate filaments.
**[[Tonofilament]]s.
**Microvilli.
*[[Spindle cell]] component - mostly features less.
**Poorly formed desmosomes.
**No intermediate filaments, no myofilaments.
 
===Molecular pathology===
Associated [[translocation]]:
*t(X;18)(p11.2;q11.2).<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/300813 http://www.ncbi.nlm.nih.gov/omim/300813]. Accessed on: 30 May 2010.</ref>
**SYT/SSX fusion gene.
 
Several SSX genes - cannot be differentiated with standard karyotyping:
*SSX1.
*SSX2 - better survival, rarely seen in biphasic tumours.<ref>{{Ref APBR|625 Q6}}</ref>
*SSX4 - uncommon.
 
Notes:
*At HSC t(X,18) = synovial sarcoma.
 
==See also==
*[[Soft tissue lesions]].
 
==References==
{{Reflist|2}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]
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