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| ==Synovial sarcoma== | | ==Synovial sarcoma== |
| *Abbreviated ''SS''.
| | {{Main|Synovial sarcoma}} |
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| ===General===
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| *Does not arise from cartilage.<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
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| *Young adults or adolescents.
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| **Classic age: 30s.
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| *Poor prognosis.
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| 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>
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| *Present with soft palpable mass - slow growing - often for years.
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| *May present with pain.
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| **Uncommon finding in sarcomas.
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| ===Gross===
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| Location:
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| *Usually close to a joint.
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| *Usually distal extremity ~65% of cases.<ref name=pmid16973781/>
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| **Upper extremity ~20% of cases.<ref name=pmid16973781/>
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| Appearance - often non-specific:
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| *Solid often lobulated +/- cystic component.
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| *Grey-yellow.
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| *Pushing border to ill-defined border.
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| Images:
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| *[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>
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| *[http://www.tumorlibrary.com/case/detail.jsp?image_id=2462 SS (tumorlibrary.com)].
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| ===Microscopic===
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| 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>
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| #Spindle cell sarcoma with features of hemangiopericytoma, i.e. [[staghorn vessels]].
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| #Biphasic synovial sarcoma:
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| ##Spindle cells with features of hemangiopericytoma.
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| ##Epitheliod glands or nests.
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| #Primitive round cell type.
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| Features:
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| *[[Herring bone]] or [[vesicular pattern]] - '''key feature'''.
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| *Spindle cells.
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| *+/-Glandular component - typically more pink.
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| *+/-Calcification - uncommon.
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| **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>
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| DDx:
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| *[[MPNST]].
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| **Can be difficult.
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| ====Images====
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| <gallery>
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| Image:Monophasic_synovial_sarcoma_-_intermed_mag.jpg | Monophasic synovial sarcoma with staghorn vessels - intermed. mag. (WC/Nephron)
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| Image:Monophasic_synovial_sarcoma_-_high_mag.jpg | Monophasic synovial sarcoma with staghorn vessels - high mag. (WC/Nephron)
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| </gallery>
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| www:
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| *[http://www.scielo.br/img/revistas/ibju/v30n3/3a06f03.jpg Synovial sarcoma (scielo.br)].
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| *[http://www.humpath.com/spip.php?page=article&id_article=1965 Synovial sarcoma - collection of images (humpath.com)].
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| *[http://path.upmc.edu/cases/case292.html Synovial sarcoma - several images (upmc.edu)].
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| *[http://radiographics.rsna.org/content/26/5/1543/F14.expansion.html Biphasic SS (radiographics.rsna.org)].
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| *[http://radiographics.rsna.org/content/26/5/1543.long Monophasic SS (radiographics.rsna.org)].
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| ===IHC===
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| Features:<ref name=Ref_WMSP627>{{Ref WMSP|627}}</ref>
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| *Vimentin +ve.
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| *EMA +ve.
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| *BCL2 +ve.
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| *CD99 +ve.
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| Others:
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| *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>
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| *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>
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| *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>
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| 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>
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| *CD34.
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| *S100 ~30% +ve.
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| *SMA.
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| Notes:
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| *Synovial sarcoma & MPNST:
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| **Both +ve: PGP9.5 (UCHL1<ref>{{OMIM|191342}}</ref>), S100, NGFR, CD56, CD99, vimentin.
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| **Synovial +ve: EMA, keratin, BCL2, TLE1.
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| **MPNST +ve: nestin, CD34.
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| Trivia:
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| *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>
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| ===EM===
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| 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>
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| *Biphasic tumour have biphasic ultrastructural features (unlike spindle cell carcinoma and [[epithelioid sarcoma]]).
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| *Epithelioid component is adenocarcinoma-like - they have:
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| **Intermediate filaments.
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| **[[Tonofilament]]s.
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| **Microvilli.
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| *[[Spindle cell]] component - mostly features less.
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| **Poorly formed desmosomes.
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| **No intermediate filaments, no myofilaments.
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| ===Molecular pathology===
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| Associated [[translocation]]:
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| *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>
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| **SYT/SSX fusion gene.
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| Several SSX genes - cannot be differentiated with standard karyotyping:
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| *SSX1.
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| *SSX2 - better survival, rarely seen in biphasic tumours.<ref>{{Ref APBR|625 Q6}}</ref>
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| *SSX4 - uncommon.
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| Notes:
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| *At HSC t(X,18) = synovial sarcoma.
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| =Other= | | =Other= |