Difference between revisions of "Synovial sarcoma"

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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]], [[hemangiopericytoma]], [[fibrosarcoma]], [[small round cell tumours]], carcinoma
| 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]]
[[Category:Soft tissue lesions]]

Latest revision as of 17:35, 23 January 2014

Synovial sarcoma
Diagnosis in short

Monophasic synovial sarcoma. H&E stain.
LM 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
LM DDx MPNST, hemangiopericytoma, fibrosarcoma, small round cell tumours, carcinoma
IHC Vimentin +ve, EMA +ve, BCL2 +ve, CD99 +ve.
Molecular t(X;18)
Gross usually lower extremity, usually close to a joint
Site soft tissue
Clinical history young adults or adolescents
Signs mass +/-pain
Prevalence uncommon
Prognosis poor

Synovial sarcoma, abbreviated SS, is an uncommon malignant soft tissue tumour, typically seen in young adults.

General

  • Does not arise from cartilage.[1]
  • Young adults or adolescents.
    • Classic age: 30s.
  • Poor prognosis.

Clinical:[2]

  • 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.[2]
    • Upper extremity ~20% of cases.[2]

Appearance - often non-specific:

  • Solid often lobulated +/- cystic component.
  • Grey-yellow.
  • Pushing border to ill-defined border.

Images:

Microscopic

Comes in three (histologic) flavours:[1][4]

  1. Spindle cell sarcoma with features of hemangiopericytoma, i.e. staghorn vessels.
  2. Biphasic synovial sarcoma:
    1. Spindle cells with features of hemangiopericytoma.
    2. Epitheliod glands or nests.
  3. 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.[5]

DDx:

Images

  • Monophasic synovial sarcoma with staghorn vessels - intermed. mag. (WC/Nephron)

  • Monophasic synovial sarcoma with staghorn vessels - high mag. (WC/Nephron)

www:

IHC

Features:[1]

  • Vimentin +ve.
  • EMA +ve.
  • BCL2 +ve.
  • CD99 +ve.

Others:

  • Beta-catenin +ve ~30-70%.[6]
  • Cyclin D1 ~50%.[6][7]
  • TLE1 +ve nuclear staining; not specific for synovial sarcoma.[8][9]

Typically negative:[10]

  • CD34.
  • S100 ~30% +ve.
  • SMA.

Notes:

  • Synovial sarcoma & MPNST:
    • Both +ve: PGP9.5 (UCHL1[11]), S100, NGFR, CD56, CD99, vimentin.
    • Synovial +ve: EMA, keratin, BCL2, TLE1.
    • MPNST +ve: nestin, CD34.

Trivia:

  • PGP in PGP9.5 = protein gene product.[12]

EM

Features:[13]

  • Biphasic tumour have biphasic ultrastructural features (unlike spindle cell carcinoma and epithelioid sarcoma).
  • Epithelioid component is adenocarcinoma-like - they have:
  • 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).[14]
    • SYT/SSX fusion gene.

Several SSX genes - cannot be differentiated with standard karyotyping:

  • SSX1.
  • SSX2 - better survival, rarely seen in biphasic tumours.[15]
  • SSX4 - uncommon.

Notes:

  • At HSC t(X,18) = synovial sarcoma.

See also

References

  1. ↑ 1.0 1.1 1.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 627. ISBN 978-0781765275.
  2. ↑ 2.0 2.1 2.2 Murphey, MD.; Gibson, MS.; Jennings, BT.; Crespo-Rodríguez, AM.; Fanburg-Smith, J.; Gajewski, DA.. "From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation.". Radiographics 26 (5): 1543-65. doi:10.1148/rg.265065084. PMID 16973781.
  3. ↑ URL: http://www.sarcomaimages.com/index.php?v=Synovial-Sarcoma. Accessed on: 2 April 2012.
  4. ↑ Schaal CH, Navarro FC, Moraes Neto FA (2004). "Primary renal sarcoma with morphologic and immunohistochemical aspects compatible with synovial sarcoma". Int Braz J Urol 30 (3): 210–3. PMID 15689250. http://www.brazjurol.com.br/may_june_2004/Schaal_ing_210_213.htm.
  5. ↑ Varela-Duran, J.; Enzinger, FM. (Jul 1982). "Calcifying synovial sarcoma.". Cancer 50 (2): 345-52. PMID 6282441.
  6. ↑ 6.0 6.1 Horvai AE, Kramer MJ, O'Donnell R (June 2006). "Beta-catenin nuclear expression correlates with cyclin D1 expression in primary and metastatic synovial sarcoma: a tissue microarray study". Arch. Pathol. Lab. Med. 130 (6): 792–8. PMID 16740029.
  7. ↑ Ng TL, Gown AM, Barry TS, et al. (January 2005). "Nuclear beta-catenin in mesenchymal tumors". Mod. Pathol. 18 (1): 68–74. doi:10.1038/modpathol.3800272. PMID 15375433.
  8. ↑ Kosemehmetoglu K, Vrana JA, Folpe AL (July 2009). "TLE1 expression is not specific for synovial sarcoma: a whole section study of 163 soft tissue and bone neoplasms". Mod. Pathol. 22 (7): 872–8. doi:10.1038/modpathol.2009.47. PMID 19363472. http://www.nature.com/modpathol/journal/v22/n7/full/modpathol200947a.html.
  9. ↑ Seo SW, Lee H, Lee HI, Kim HS (February 2011). "The role of TLE1 in synovial sarcoma". J Orthop Res. doi:10.1002/jor.21318. PMID 21319215.
  10. ↑ URL: http://path.upmc.edu/cases/case292/dx.html. Accessed on: 14 January 2012.
  11. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 191342
  12. ↑ Doran, JF.; Jackson, P.; Kynoch, PA.; Thompson, RJ. (Jun 1983). "Isolation of PGP 9.5, a new human neurone-specific protein detected by high-resolution two-dimensional electrophoresis.". J Neurochem 40 (6): 1542-7. PMID 6343558.
  13. ↑ Fisher, C. (Dec 1998). "Synovial sarcoma.". Ann Diagn Pathol 2 (6): 401-21. PMID 9930576.
  14. ↑ URL: http://www.ncbi.nlm.nih.gov/omim/300813. Accessed on: 30 May 2010.
  15. ↑ Lefkowitch, Jay H. (2006). Anatomic Pathology Board Review (1st ed.). Saunders. pp. 625 Q6. ISBN 978-1416025887.
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