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Synovial sarcoma

February 28, 2023

A synovial sarcoma (also known as malignant synovioma[1]) is a rare form of cancer which occurs primarily in the extremities of the arms or legs, often in proximity to joint capsules and tendon sheaths.[2] It is a type of soft-tissue sarcoma.

Synovial sarcoma
Other namesMalignant synovioma
Micrograph of a monophasic synovial sarcoma. The histologic appearance is non-specific and overlaps with MPNST and fibrosarcoma. H&E stain.
SpecialtyOncology

The name "synovial sarcoma" was coined early in the 20th century, as some researchers thought that the microscopic similarity of some tumors to synovium, and its propensity to arise adjacent to joints, indicated a synovial origin; however, the actual cells from which the tumor develops are unknown and not necessarily synovial.[3]

Primary synovial sarcomas are most common in the soft tissue near the large joints of the arm and leg but have been documented in most human tissues and organs, including the brain, prostate, and heart.

Synovial sarcoma occurs in about 1–2 per 1,000,000 people a year.[4] They occur most commonly in the third decade of life, with males being affected more often than females (ratio around 1.2:1).[4][2]

Signs and symptoms

Synovial sarcoma usually presents with an otherwise asymptomatic swelling or mass, although general symptoms related to malignancies can be reported such as fatigue.[5]

Diagnosis

The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18) chromosomal translocation.[6]

Histopathology

Two cell types can be seen microscopically in synovial sarcoma. One fibrous type, known as a spindle or sarcomatous cell, is relatively small and uniform, and found in sheets. The other is epithelial in appearance. Classical synovial sarcoma has a biphasic appearance with both types present. Synovial sarcoma can also appear to be poorly differentiated or to be monophasic fibrous, consisting only of sheets of spindle cells. Some authorities[3] state that, extremely rarely, there can be a monophasic epithelial form which causes difficulty in differential diagnosis. Depending on the site, there is similarity to biphenotypic sinonasal sarcoma, although the genetic findings are distinctive.

Like other soft tissue sarcomas, there is no universal grading system for reporting histopathology results.[7] In Europe, the Trojani or French system is gaining in popularity[8] while the NCI grading system is more common in the United States. The Trojani system scores the sample, depending on tumour differentiation, mitotic index, and tumour necrosis, between 0 and 6 and then converts this into a grade of between 1 and 3, with 1 representing a less aggressive tumour.[7] The NCI system is also a three-grade one, but takes a number of other factors into account.

Immunohistochemistry (IHC): SS18-fusion specific antibody and SSX-CT antibody are highly sensitive and specific for synovial sarcoma and when used together may obviate the need for molecular testing in most cases.[9][10] Cytokeratin is typically expressed, at least focally. TLE1, BCL2 and CD99 may be positive but lack specificity.

Molecular biology

Most, and perhaps all, cases of synovial sarcoma are associated with a reciprocal translocation t(x;18)(p11.2;q11.2). There is some debate about whether the molecular observation itself is definitive of synovial sarcoma.[11][12][13]

The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).[6] This translocation event between the SS18 gene on chromosome 18 and one of 3 SSX genes (SSX1, SSX2 and SSX4) on chromosome X causes the presence of an SS18-SSX fusion gene. The resulting fusion protein brings together the transcriptional activating domain of SS18 and the transcriptional repressor domains of SSX. It also incorporates into the SWI/SNF chromatin remodeling complex, a well known tumor suppressor.[14] SS18-SSX is thought to underlie synovial sarcoma pathogenesis through dysregulation of gene expression.[3]

There is some association between the SS18-SSX1 or SS18-SSX2 fusion type and both tumour morphology and five-year survival.[15]

Treatment

Treatment is usually multimodal, involving surgery, chemotherapy and radiotherapy:[16]

  • Surgery, to remove the tumor and a safety margin of healthy tissue. This is the mainstay of synovial sarcoma treatment and is curative in approximately 20–70% of patients, depending on the particular study being quoted.[17]
  • Conventional chemotherapy, (for example, doxorubicin hydrochloride and ifosfamide), to reduce the number of remaining microscopic metastases.[16] The benefit of chemotherapy in synovial sarcoma to overall survival remains unclear, although a recent study has shown that survival of patients with advanced, poorly differentiated disease marginally improves with doxorubicin/ifosfamide treatment.[16][18]
  • Radiotherapy to reduce the chance of local recurrence.[16] The benefit of radiotherapy in this disease is less clear than for chemotherapy.[16]

References

  1. ^ "Synovioma". Encyclopædia Britannica Online. Retrieved 20 May 2012.
  2. ^ a b Goldblum, John R.; Folpe, Andrew L.; Weiss, Sharon W. (2001). "33". Enzinger and Weiss's Soft Tissue Tumors (Sixth ed.). St Louis, Missouri: CV Mosby. pp. 1052–1070. ISBN 978-0323088343. LCCN 2013010770.
  3. ^ a b c Pollock, Raphael E., ed. (2002). Soft Tissue Sarcomas. American Cancer Society Atlas of Clinical Oncology. BC Decker. ISBN 155009128X.
  4. ^ a b Ferrari and Collini (2012). "Synovial Sarcoma". ESUN. 9 (5).
  5. ^ 楊照彬 (2010). "青少年骨髓性肉瘤初期以背痛呈現: 病例報告". 台灣復健醫學雜誌 (in Chinese). 38 (4): 269–275.
  6. ^ a b Coindre, Jean-Michel; Pelmus, Manuela; Hostein, Isabelle; Lussan, Catherine; Bui, Binh N.; Guillou, Louis (2003). "Should molecular testing be required for diagnosing synovial sarcoma? A prospective study of 204 cases". Cancer. 98 (12): 2700–7. doi:10.1002/cncr.11840. PMID 14669292.
  7. ^ a b Coindre, Jean-Michel (October 2006). "Grading of soft tissue sarcomas: review and update". Archives of Pathology & Laboratory Medicine. 130 (10): 1448–53. doi:10.5858/2006-130-1448-GOSTSR. PMID 17090186.
  8. ^ Paul, A.S.; Charalambous, C.; Maltby, B.; Whitehouse, R. (April 2003). "The management of soft-tissue sarcomas of the extremities". Current Orthopaedics. 17 (2): 124–133. doi:10.1054/cuor.2002.0314. ISSN 0268-0890.
  9. ^ Gill, Anthony J.; Zaborowski, Matthew; Vargas, Ana C.; et al. (19 June 2020). "When used together SS18–SSX fusion‐specific and SSX C‐terminus immunohistochemistry are highly specific and sensitive for the diagnosis of synovial sarcoma and can replace FISH or molecular testing in most cases". Histopathology. 77 (4): 588–600. doi:10.1111/his.14190. PMID 32559341. S2CID 219949018.
  10. ^ Baranov, Esther; McBride, Matthew J.; Bellizzi, Andrew M.; Ligon, Azra Hadi; Fletcher, Christopher D. M.; Kadoch, Cigall; Hornick, Jason L. (July 2020). "A Novel SS18-SSX Fusion-specific Antibody for the Diagnosis of Synovial Sarcoma". The American Journal of Surgical Pathology. 44 (7): 922–33. doi:10.1097/PAS.0000000000001447. PMC 7289668. PMID 32141887.
  11. ^ Pfeifer, John D.; Hill, D. Ashley; O'Sullivan, Maureen J.; Dehner, Louis P. (4 January 2002). "Diagnostic gold standard for soft tissue tumours: morphology or molecular genetics?". Histopathology. 37 (6): 485–500. doi:10.1046/j.1365-2559.2000.01107.x. PMID 11122430. S2CID 6825413.
  12. ^ O'Sullivan, Maureen J.; Kyriakos, M.; Zhu, X.; Wick, M.R.; Swanson, P.E.; Dehner, Louis P.; Humphrey, P.A.; Pfeifer, John D. (2000). "Malignant peripheral nerve sheath tumors with t(X;18). A pathologic and molecular genetic study". Modern Pathology. 13 (12): 1336–46. doi:10.1038/modpathol.3880247. PMID 11144931.
  13. ^ Coindre, Jean-Michel; Hostein, Isabelle; Benhattar, Jean; Lussan, Cathy; Rivel, Janine; Guillou, Louis (June 2002). "Malignant Peripheral Nerve Sheath Tumors are t(X;18)-Negative Sarcomas. Molecular Analysis of 25 Cases Occurring in Neurofibromatosis Type 1 Patients, Using Two Different RT-PCR-Based Methods of Detection". Modern Pathology. 15 (6): 589–92. doi:10.1038/modpathol.3880570. PMID 12065770.
  14. ^ Middeljans, Evelien; Wan, Xi; Jansen, Pascal W.; Sharma, Vikram; Stunnenberg, Hendrik G.; Logie, Colin (March 2012). Freitag, Michael (ed.). "SS18 together with animal-specific factors defines human BAF-type SWI/SNF complexes". PLOS One. 7 (3): e33834. Bibcode:2012PLoSO...733834M. doi:10.1371/journal.pone.0033834. PMC 3307773. PMID 22442726.
  15. ^ Ladanyi, Marc; Antonescu, Christina R.; Leung, Denis H.Y.; et al. (January 2002). "Impact of SS18-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients" (PDF). Cancer Research. 62 (1): 135–40. PMID 11782370. (PDF) from the original on 26 August 2017.
  16. ^ a b c d e Thway, Khin; Fisher, Cyril (December 2014). "Synovial sarcoma: defining features and diagnostic evolution". Annals of Diagnostic Pathology. 18 (6): 369–380. doi:10.1016/j.anndiagpath.2014.09.002. ISSN 1092-9134. PMID 25438927.
  17. ^ Lewis, Jonathan J.; Antonescu, Cristina R.; Leung, Denis Hy.Y.; Blumberg, David; Healey, John H.; Woodruff, James M.; Brennan, Murray F. (2000). "Synovial sarcoma: a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity". Journal of Clinical Oncology. 18 (10): 2087–94. doi:10.1200/JCO.2000.18.10.2087. PMID 10811674.
  18. ^ Ren, Xiao-Hua; WU, Xiao-Min; JIN, Cheng; CUI, Yong-An (2009). "Advances in the diagnosis and treatment of synovial sarcoma". Journal of Medical Biomechanics (in Chinese). 15 (4): 541–542. Retrieved 7 May 2016.

External links

  •   Media related to Synovial sarcoma at Wikimedia Commons
  • Sarcoma Help: What is Synovial Sarcoma?
  • WebMD: Synovial Sarcoma Overview

February 28, 2023
synovial, sarcoma, synovial, sarcoma, also, known, malignant, synovioma, rare, form, cancer, which, occurs, primarily, extremities, arms, legs, often, proximity, joint, capsules, tendon, sheaths, type, soft, tissue, sarcoma, other, namesmalignant, synoviomamic. A synovial sarcoma also known as malignant synovioma 1 is a rare form of cancer which occurs primarily in the extremities of the arms or legs often in proximity to joint capsules and tendon sheaths 2 It is a type of soft tissue sarcoma Synovial sarcomaOther namesMalignant synoviomaMicrograph of a monophasic synovial sarcoma The histologic appearance is non specific and overlaps with MPNST and fibrosarcoma H amp E stain SpecialtyOncologyThe name synovial sarcoma was coined early in the 20th century as some researchers thought that the microscopic similarity of some tumors to synovium and its propensity to arise adjacent to joints indicated a synovial origin however the actual cells from which the tumor develops are unknown and not necessarily synovial 3 Primary synovial sarcomas are most common in the soft tissue near the large joints of the arm and leg but have been documented in most human tissues and organs including the brain prostate and heart Synovial sarcoma occurs in about 1 2 per 1 000 000 people a year 4 They occur most commonly in the third decade of life with males being affected more often than females ratio around 1 2 1 4 2 Contents 1 Signs and symptoms 2 Diagnosis 2 1 Histopathology 2 2 Molecular biology 3 Treatment 4 References 5 External linksSigns and symptoms EditSynovial sarcoma usually presents with an otherwise asymptomatic swelling or mass although general symptoms related to malignancies can be reported such as fatigue 5 Diagnosis EditThe diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t X 18 chromosomal translocation 6 Histopathology Edit Two cell types can be seen microscopically in synovial sarcoma One fibrous type known as a spindle or sarcomatous cell is relatively small and uniform and found in sheets The other is epithelial in appearance Classical synovial sarcoma has a biphasic appearance with both types present Synovial sarcoma can also appear to be poorly differentiated or to be monophasic fibrous consisting only of sheets of spindle cells Some authorities 3 state that extremely rarely there can be a monophasic epithelial form which causes difficulty in differential diagnosis Depending on the site there is similarity to biphenotypic sinonasal sarcoma although the genetic findings are distinctive Like other soft tissue sarcomas there is no universal grading system for reporting histopathology results 7 In Europe the Trojani or French system is gaining in popularity 8 while the NCI grading system is more common in the United States The Trojani system scores the sample depending on tumour differentiation mitotic index and tumour necrosis between 0 and 6 and then converts this into a grade of between 1 and 3 with 1 representing a less aggressive tumour 7 The NCI system is also a three grade one but takes a number of other factors into account Immunohistochemistry IHC SS18 fusion specific antibody and SSX CT antibody are highly sensitive and specific for synovial sarcoma and when used together may obviate the need for molecular testing in most cases 9 10 Cytokeratin is typically expressed at least focally TLE1 BCL2 and CD99 may be positive but lack specificity Molecular biology Edit Most and perhaps all cases of synovial sarcoma are associated with a reciprocal translocation t x 18 p11 2 q11 2 There is some debate about whether the molecular observation itself is definitive of synovial sarcoma 11 12 13 The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t X 18 6 This translocation event between the SS18 gene on chromosome 18 and one of 3 SSX genes SSX1 SSX2 and SSX4 on chromosome X causes the presence of an SS18 SSX fusion gene The resulting fusion protein brings together the transcriptional activating domain of SS18 and the transcriptional repressor domains of SSX It also incorporates into the SWI SNF chromatin remodeling complex a well known tumor suppressor 14 SS18 SSX is thought to underlie synovial sarcoma pathogenesis through dysregulation of gene expression 3 There is some association between the SS18 SSX1 or SS18 SSX2 fusion type and both tumour morphology and five year survival 15 Treatment EditTreatment is usually multimodal involving surgery chemotherapy and radiotherapy 16 Surgery to remove the tumor and a safety margin of healthy tissue This is the mainstay of synovial sarcoma treatment and is curative in approximately 20 70 of patients depending on the particular study being quoted 17 Conventional chemotherapy for example doxorubicin hydrochloride and ifosfamide to reduce the number of remaining microscopic metastases 16 The benefit of chemotherapy in synovial sarcoma to overall survival remains unclear although a recent study has shown that survival of patients with advanced poorly differentiated disease marginally improves with doxorubicin ifosfamide treatment 16 18 Radiotherapy to reduce the chance of local recurrence 16 The benefit of radiotherapy in this disease is less clear than for chemotherapy 16 References Edit Synovioma Encyclopaedia Britannica Online Retrieved 20 May 2012 a b Goldblum John R Folpe Andrew L Weiss Sharon W 2001 33 Enzinger and Weiss s Soft Tissue Tumors Sixth ed St Louis Missouri CV Mosby pp 1052 1070 ISBN 978 0323088343 LCCN 2013010770 a b c Pollock Raphael E ed 2002 Soft Tissue Sarcomas American Cancer Society Atlas of Clinical Oncology BC Decker ISBN 155009128X a b Ferrari and Collini 2012 Synovial Sarcoma ESUN 9 5 楊照彬 2010 青少年骨髓性肉瘤初期以背痛呈現 病例報告 台灣復健醫學雜誌 in Chinese 38 4 269 275 a b Coindre Jean Michel Pelmus Manuela Hostein Isabelle Lussan Catherine Bui Binh N Guillou Louis 2003 Should molecular testing be required for diagnosing synovial sarcoma A prospective study of 204 cases Cancer 98 12 2700 7 doi 10 1002 cncr 11840 PMID 14669292 a b Coindre Jean Michel October 2006 Grading of soft tissue sarcomas review and update Archives of Pathology amp Laboratory Medicine 130 10 1448 53 doi 10 5858 2006 130 1448 GOSTSR PMID 17090186 Paul A S Charalambous C Maltby B Whitehouse R April 2003 The management of soft tissue sarcomas of the extremities Current Orthopaedics 17 2 124 133 doi 10 1054 cuor 2002 0314 ISSN 0268 0890 Gill Anthony J Zaborowski Matthew Vargas Ana C et al 19 June 2020 When used together SS18 SSX fusion specific and SSX C terminus immunohistochemistry are highly specific and sensitive for the diagnosis of synovial sarcoma and can replace FISH or molecular testing in most cases Histopathology 77 4 588 600 doi 10 1111 his 14190 PMID 32559341 S2CID 219949018 Baranov Esther McBride Matthew J Bellizzi Andrew M Ligon Azra Hadi Fletcher Christopher D M Kadoch Cigall Hornick Jason L July 2020 A Novel SS18 SSX Fusion specific Antibody for the Diagnosis of Synovial Sarcoma The American Journal of Surgical Pathology 44 7 922 33 doi 10 1097 PAS 0000000000001447 PMC 7289668 PMID 32141887 Pfeifer John D Hill D Ashley O Sullivan Maureen J Dehner Louis P 4 January 2002 Diagnostic gold standard for soft tissue tumours morphology or molecular genetics Histopathology 37 6 485 500 doi 10 1046 j 1365 2559 2000 01107 x PMID 11122430 S2CID 6825413 O Sullivan Maureen J Kyriakos M Zhu X Wick M R Swanson P E Dehner Louis P Humphrey P A Pfeifer John D 2000 Malignant peripheral nerve sheath tumors with t X 18 A pathologic and molecular genetic study Modern Pathology 13 12 1336 46 doi 10 1038 modpathol 3880247 PMID 11144931 Coindre Jean Michel Hostein Isabelle Benhattar Jean Lussan Cathy Rivel Janine Guillou Louis June 2002 Malignant Peripheral Nerve Sheath Tumors are t X 18 Negative Sarcomas Molecular Analysis of 25 Cases Occurring in Neurofibromatosis Type 1 Patients Using Two Different RT PCR Based Methods of Detection Modern Pathology 15 6 589 92 doi 10 1038 modpathol 3880570 PMID 12065770 Middeljans Evelien Wan Xi Jansen Pascal W Sharma Vikram Stunnenberg Hendrik G Logie Colin March 2012 Freitag Michael ed SS18 together with animal specific factors defines human BAF type SWI SNF complexes PLOS One 7 3 e33834 Bibcode 2012PLoSO 733834M doi 10 1371 journal pone 0033834 PMC 3307773 PMID 22442726 Ladanyi Marc Antonescu Christina R Leung Denis H Y et al January 2002 Impact of SS18 SSX fusion type on the clinical behavior of synovial sarcoma a multi institutional retrospective study of 243 patients PDF Cancer Research 62 1 135 40 PMID 11782370 Archived PDF from the original on 26 August 2017 a b c d e Thway Khin Fisher Cyril December 2014 Synovial sarcoma defining features and diagnostic evolution Annals of Diagnostic Pathology 18 6 369 380 doi 10 1016 j anndiagpath 2014 09 002 ISSN 1092 9134 PMID 25438927 Lewis Jonathan J Antonescu Cristina R Leung Denis Hy Y Blumberg David Healey John H Woodruff James M Brennan Murray F 2000 Synovial sarcoma a multivariate analysis of prognostic factors in 112 patients with primary localized tumors of the extremity Journal of Clinical Oncology 18 10 2087 94 doi 10 1200 JCO 2000 18 10 2087 PMID 10811674 Ren Xiao Hua WU Xiao Min JIN Cheng CUI Yong An 2009 Advances in the diagnosis and treatment of synovial sarcoma Journal of Medical Biomechanics in Chinese 15 4 541 542 Retrieved 7 May 2016 External links Edit Media related to Synovial sarcoma at Wikimedia Commons Sarcoma Help What is Synovial Sarcoma WebMD Synovial Sarcoma Overview Retrieved from https en wikipedia org w index php title Synovial sarcoma amp oldid 1063752943, wikipedia, wiki, book, books, library,

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