Abstract
Synovial sarcoma is an aggressive soft tissue sarcoma of uncertain histogenesis with a predilection for the deep soft tissues of paraarticular regions of the extremities. The tumor predominantly develops in younger adults and accounts for 5–10 % of all soft tissue sarcomas. Apart from extremities, this sarcoma also occurs in various other sites, including visceral organs, the heart and large blood vessels. Most instances of hepatic manifestations of this neoplasm are metastases. However, few cases of monophasic primary hepatic synovial sarcoma have been reported. The tumors are sometimes large and hemorrhagic. Histologically, spindle cells and hemangiopericytoma-like cells prevail, with variable admixtures of epithelioid cells. The tumors are associated with the typical SYT-SSX1 or SYT-SSX2 rearrangement and the corresponding fusion proteins, as in soft tissue synovial sarcomas. Very rarely, synovial sarcoma develops as a primary lesion in the biliary tract.
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References
Allander SV, Illei PB, Chen Y, Antonescu CR, Bittner M, Ladanyi M, Meltzer PS (2002) Expression profiling of synovial sarcoma by cDNA microarrays: association of ERBB2, IGFBP2, and ELF3 with epithelial differentiation. Am J Pathol 161:1531–1534
Antonescu CR, Kawai A, Leung DH, Lonardo F, Woodruff JM, Healey JH, Ladanyi M (2000) Strong association of SYT-SSX fusion type and morphologic epithelial differentiation in synovial sarcoma. Diagn Mol Pathol 9:1–8
Barco R, Hunt LB, Frump AL, Garcia CB, Benesh A, Caldwell RL, Eid JE (2007) The synovial sarcoma SYT-SSX2 oncogene remodels the cytoskeleton through activation of the ephrin pathway. Mol Biol Cell 18:4003–4012
Barco R, Garcia CB, Eid JE (2009) The synovial sarcoma-associated SYT-SSX2 oncogene antagonizes the polycomb complex protein Bmi1. PLoS One 4:e5060
Billings SD, Meisner LF, Cummings OW, Tejada E (2000) Synovial sarcoma of the upper digestive tract: a report of two cases with demonstration of the X;18 translocation by fluorescence in situ hybridization. Mod Pathol 13:68–76
Chu PG, Benhattar J, Weiss LM, Meaghar-Villemure K (2004) Intraneural synovial sarcoma: two cases. Mod Pathol 17:258–263
Company Campins M.M., Morales R, Dolz C, Garcia-Bonafe M, Vilella A, Huguet P (2009) Primary monophasic synovial sarcoma of the duodenum confirmed by cytogenetic analysis with demonstration of t(X;18): a case report. J Gastrointestin Liver Dis 18:89–93
De Torres C, Cardesa TM, Rodriguez-Perales S, Cigudosa JC, Mora J (2005) Epithelioid sarcoma with SYT-SSX1 fusion gene expression: molecular and cytogenetic analysis. Cancer Genet Cytogenet 162:50–56
Dormeyer W, Ott M, Schnolzer M (2005) Analysis of p300 acetyltransferase substrate specificity by MALDI TOF mass spectrometry. Methods 36:376–382
Eid JE, Kung AL, Scully R, Livingstone DM (2000) p300 interacts with the nuclear proto-oncoprotein SYT as part of the active control of cell adhesion. Cell 102:839–848
Ferrari A, De Salvo GL, Brennan B, van Noesel MM, De Paoli A, Casanova M, Francotte N et al (2015) Synovial sarcoma in children and adolescents: the European Pediatric Soft Tissue Sarcoma Study Group prospective trial ( EpSSG NRSTS 2005). Ann Oncol 26:567–572
Fisher C, Folpe AL, Hashimoto H, Weiss SW (2004) Intra-abdominal synovial sarcoma: a clinicopathological study. Histopathology 45:245–253
Fukukawa C, Nagayama S, Tsunoda T, Toguchida J, Nakamura Y, Katagiri T (2009) Activation of the non-canonical Dvl-Rac1-JNK pathway by Frizzled homologue 10 in human synovial sarcoma. Oncogene 28:1110–1120
Fukuoka K (2006) Molecular detection of SYT-SSX fusion gene transcripts currently represents the most specific and sensitive tool for diagnosing intrathoracic synovial sarcoma. Intern Med 45:881–882
Furumatsu T, Tsuda M, Yoshida K, Taniguchi N, Ito T, Hashimoto M, Ito T, Asahara H (2005) Sox9 and p300 cooperatively regulate chromatin-mediated transcription. J Biol Chem 280:35203–35208
Guillou L, Benhattar J, Bonichou F, Gallagher G, Terrier P, Stauffer E, Somerhausen NS et al (2004) Histologic grade, but not SYT-SSX fusion type, is an important prognostic factor in patients with synovial sarcoma: a multicenter, retrospective analysis. J Clin Oncol 22:4040–4050
Haldar M, Randall RL, Capecchi MR (2008) Synovial sarcoma: from genetics to genetic-based animal modeling. Clin Orthop Relat Res 466:2156–2167
Hara S, Hatori M, Hosaka M, Komatsu T, Tsuchiya T, Kimura N (2003) Synovial sarcoma with massive ossification – a case report. Ups J Med Sci 108:239
Holla P, Hafez GR, Slukvin I, Kalayoglu M (2006) Synovial sarcoma, a primary liver tumor – a case report. Pathol Res Pract 202:385–387
Horvai AE, Kramer MJ, O’Donnell R (2006) Beta-catenin nuclear expression correlates with cyclin D1 expression in primary and metastatic synovial sarcoma: a tissue microarray study. Arch Pathol Lab Med 130:792–798
Huang WC, Chen CC (2005) Akt phosphorylation of p300 at Ser-1834 is essential for its histone acetyltransferase and transcriptional activity. Mol Cell Biol 25:6592–6602
Ito T, Ouchida M, Ito S, Jitsumori Y, Morimoto Y, Ozaki T, Kawai A, Inoue H, Shimizu K (2004) SYT, a partner of SYT-SSX oncoprotein in synovial sarcomas, interacts with mSin3A, a component of histone deacetylase complex. Lab Invest 84:1484–1490
Iwasaki T, Koibuchi N, Chin WW (2005) Synovial sarcoma translocation ( SYT ) encodes a nuclear receptor coactivator. Endocrinology 146:3892–3899
Jun SY, Choi J, Kang GH, Park SH, Avala AG, Ro JY (2004) Synovial sarcoma of the kidney with rhabdoid features: report of three cases. Am J Surg Pathol 28:634–637
Kato H, Tjernberg A, Zhang W, Krutchinsky AN, An W, Takeuchi T, Ohtsuki Y, Sugano S et al (2002) SYT associates with human SNF/SWI complexes and the C-terminal region of its fusion partner SSX1 targets histones. J Biol Chem 277:5498–5505
Kawai A, Woodruff J, Healey JH, Brennan MF, Antonescu CR, Ladanyi M (1998) SYT-SSX gene fusion as a determinant of morphology and prognosis in synovial sarcoma. N Engl J Med 338:153–160
Kerouanton A, Jimenez I, Cellier C, Laurence V, Helfre S, Pannier S, Mary P, Freneaux P et al (2014) Synovial sarcoma in children and adolescents. J Pediatr Hematol Oncol 36:257–262
Kim J, Swee M, Parks WC (2009) Cytosolic SYT/SS18 isoforms are actin-associated proteins that function in matrix-specific adhesion. PLoS One 4:e6455
Kimura T, Sakai M, Tabu K, Wang L, Tsunematsu R, Tsuda M, Sawa H, Nagashima K et al (2009) Human synovial sarcoma proto-oncogene Syt is essential for early embryonic development through the regulation of cell migration. Lab Invest 89:645–656
Knösel T, Heretsch S, Altendorf-Hofmann A, Richter P, Katenkamp K, Katenkamp D, Berndt A, Petersen I (2010) TLE1 is a robust diagnostic biomarker for synovial sarcomas and correlates with t(X;18): analysis of 319 cases. Eur J Cancer 46:1170–1176
Ko SF, Chou FF, Huang CH, Ng SH, Wan YL, Lee TY, Lin JW et al (1998) Primary synovial sarcoma of the gastrocolic ligament. Br J Radiol 71:438–440
Kohashi K, Yamamoto H, Tamiya S, Matono H, Iwamoto Y, Taguchi T, Tsuneyoshi M (2010) Reduced expression of SMARCB1/INI1 protein in synovial sarcoma. Mod Pathol 23:981–990
Köse D, Annagür A, Erol C, Ugras S, Köksal Y (2014) Synovial sarcoma in a premature newborn. Pediatr Int 56:e17–e20
Krane JF, Bertoni F, Fletcher CD (1999) Myxoid synovial sarcoma: an underappreciated morphologic subset. Mod Pathol 12:456–462
Ladanyi M, Antonescu CR, Leung DH et al (2002) Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res 62:135–140
Laskin WB, Miettinen M (2002) Epithelial-type and neural-type cadherin expression in malignant noncarcinomatous neoplasms with epithelioid features that involve the soft tissues. Arch Pathol Lab Med 126:425–431
Lazar A, Abruzzo LV, Pollock RE, Lee S, Czerniak B (2006) Molecular diagnosis of sarcomas: chromosomal translocations in sarcomas. Arch Pathol Lab Med 130:1199–1207
Liu YN, Lee WW, Wang CY, Chao TH, Chen Y, Chen JH (2005) Regulatory mechanisms controlling human E-cadherin gene expression. Oncogene 24:8277–8290
Makhlouf HR, Ahrens W, Agarwal B, Dow N, Marshalleck JJ, Lee EL, Dotto JE, Hui P et al (2008) Synovial sarcoma of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 10 cases. Am J Surg Pathol 32:275–281
Masui F, Matsuno Y, Yokoyama R, Nakanishi Y, Hasegawa T, Kanai Y, Beppu Y, Hirohashi S et al (1999) Synovial sarcoma, histologically mimicking primitive neuroectodermal tumor/Ewing’s sarcoma at distant sites. Jpn J Clin Oncol 29:438–441
Naka N, Takenaka S, Araki N, Miwa T, Hashimoto N, Yoshioka K, Joyama S, Hamada KI et al (2010) Synovial sarcoma is a stem cell malignancy. Stem Cells 28:1119–1131
Ng TL, Gown AM, Barry TS, Cheang MC, Chan AK, Turbin DA, Hsu FD, West RB, Nielsen TO (2005) Nuclear beta-catenin in mesenchymal tumors. Mod Pathol 18:68–74
Nielsen TO, Poulin NM, Ladanyi M (2015) Synovial sarcoma: recent discoveries as a roadmap to new avenues for therapy. Cancer Discov 5:124–134
Nuciforo PG, Pellegrini C, Fasani R, Maggioni M, Coggi G, Parafioriti A, Bosari S (2003) Molecular and immunohistochemical analysis of HER2/neu oncogene in synovial sarcoma. Hum Pathol 34:639–645
Perani M, Antonson P, Hamoudi R, Ingram CJ, Cooper CS, Garrett MD, Goodwin GH (2005) The proto-oncoprotein SYT interacts with SIP/CoAA a human nuclear receptor co-activator with similarity to EWS and TLS/FUS family of proteins. J Biol Chem 280:42863–42876
Pretto D, Barco R, Rivera J, Neel N, Gustavson MD, Eid JE (2006) The synovial sarcoma translocation protein SYT-SSX2 recruits beta-catenin to the nucleus and associates it in an active complex. Oncogene 25:3661–3669
Qayum A, Riall TS, Srinivasan R, Salazar FA (2008) A patient with synovial sarcoma primary to the gallbladder and common bile duct. J Gastrointest Surg 12:609–611
Rao L, Jaiprakash P, Palankar N, Gowda V (2013) Intra-abdominal primary monophasic synovial sarcoma with hemangiopericytoma-like areas. J Cancer Res Ther 9:102–104
Rekhi B, Vogel U (2015) Utility of characteristic ‘weak to absent’ INI1/SMARCB1/BAF47 expression in diagnosis of synovial sarcomas. APMIS. doi:10.1111/apm.12395
Romeo S, Rossi S, Acosta MM, Canal F, Sbaraglia M, Laurino L, Mazzoleni G et al (2015) Primary synovial sarcoma (SS) of the digestive system: a molecular and clinicopathological study of fifteen cases. Clin Sarcoma Res 5:7
Saito T, Oda Y, Sakamoto A, Tamiya S, Kinukawa N, Hayashi K, Iwamoto Y, Tsuneyoshi M (2000) Prognostic values of the preserved expression of the E-cadherin and catenin families of adhesion molecules and of beta-catenin mutations in synovial sarcoma. J Pathol 192:342–350
Saito T, Oda Y, Sugimachi K, Kawaguchi K, Tamiya S, Tanaka K, Matsuda S, Sakamoto A et al (2001) E-cadherin gene mutations frequently occur in synovial sarcoma as a determinant of histological features. Am J Pathol 159:2117–2124
Saito T, Oda Y, Sakamoto A, Tamiya S, Iwamoto Y, Tsuneyoshi M (2002) Matrix metalloproteinase-2 expression correlates with morphological and immunohistochemical epithelial characteristics in synovial sarcoma. Histopathology 40:279–285
Saito T, Oda Y, Kawaguchi K, Sugimachi K, Yamamoto H, Tateishi N, Tanaka K, Matsuda S et al (2004) E-cadherin mutation and Snail overexpression as alternative mechanisms of E-cadherin inactivation in synovial sarcoma. Oncogene 23:8629–8638
Saito T, Oda Y, Yamamoto H, Kawaguchi K, Tanaka K, Matsuda S, Iwamoto Y, Tsuneyoshi M (2006) Nuclear beta-catenin correlates with cyclin D1 expression in spindle and pleomorphic sarcomas but not in synovial sarcoma. Hum Pathol 37:689–697
Sapi Z, Papai Z, Hruska A, Antal I, Bodo M, Orosz Z (2005) Her-2 oncogene amplification, chromosome 17 and DNA ploidy status in synovial sarcoma. Pathol Oncol Res 11:133–138
Sato H, Hasegawa T, Abe Y, Sakai H, Hirohashi S (1999) Expression of E-cadherin in bone and soft tissue sarcomas: a possible role in epithelial differentiation. Hum Pathol 30:1344–1349
Sato H, Hasegawa T, Kanai Y, Tsutsumi Y, Osamura Y, Abe Y, Sakai H, Hirohashi S (2001) Expression of cadherins and their undercoat proteins ( alpha-, beta-, and gamma-catenins and p120) and accumulation of beta-catenin with no gene mutations in synovial sarcoma. Virchows Arch 438:23–30
Shaw GR, Lais CJ (1993) Fatal intravascular synovial sarcoma in a 31-year-old woman. Hum Pathol 24:809–810
Shmookler BM (1982) Retroperitoneal synovial sarcoma: a report of four cases. Am J Clin Pathol 77:669–673
Soole F, Maupain C, Defachelles AS, Taque S, Minard-Colin V, Bergeron C, De Rycke Y et al (2014) Synovial sarcoma relapses in children and adolescents: prognostic factors, treatment, and outcome. Pediatr. Blood Cancer 61:1387–1393
Srivastava A, Nielsen PG, Dal CP, Rosenberg AE (2005) Monophasic synovial sarcoma of the liver. Arch Pathol Lab Med 129:1047–1049
Su L, Cheng H, Sampaio AV, Nielsen TO, Underhill TM (2010) EGR1 reactivation by histone deacetylase inhibitors promotes synovial sarcoma cell death through the PTEN tumor suppressor. Oncogene 29:4352–4361
Sun Y, Gao D, Liu Y, Huang J, Lessnick S, Tanaka S (2006) IGF2 is critical for tumorigenesis by synovial sarcoma oncoprotein SYT-SSX1. Oncogene 25:1042–1052
Sun B, Sun Y, Wang J, Zhao X, Zhang S, Liu Y, Li X, Feng Y, Zhou H, Hao X (2008) The diagnostic value of SYT-SSX detected by reverse transcriptase-polymerase chain reaction ( RT-PCR ) and fluorescence in situ hybridization ( FISH ) for synovial sarcoma: a review and prospective study of 255 cases. Cancer Sci 99:1355–1361
Takenaka S, Ueda T, Naka N, Araki N, Hashimoto N, Myoui A, Ozaki T, Nakayama T et al (2008) Prognostic implication of SYT-SSX fusion type in synovial sarcoma: a multi-institutional retrospective analysis in Japan. Oncol Rep 19:467–476
Takenaka S, Naka N, Araki N, Hashimoto N, Ueda T, Yoshioka K, Yoshikawa H, Itoh K (2010) Downregulation of SS18-SSX1 expression in synovial sarcoma by small interfering RNA enhances the focal adhesion pathway and inhibits anchorage-independent growth in vitro and tumor growth in vivo. Int J Oncol 36:823–831
Ten Heuvel SE, Hoekstra HJ, Bastiaannet E, Suurmeijer AJ (2009) The classic prognostic factors tumor stage, tumor size, and tumor grade are the strongest predictors of outcome in synovial sarcoma: no role for SSX fusion type or ezrin expression. Appl Immunohistochem Mol Morphol 17:189–195
Thomas DG, Giordano TJ, Sanders D, Biermann S, Sondak VK, Trent JC, Yu D et al (2005) Expression of receptor tyrosine kinases epidermal growth factor receptor and HER-2/neu in synovial sarcoma. Cancer 103:830–838
Thway K, Fisher C (2014) Synovial sarcoma: defining features and diagnostic evolution. Ann Diagn Pathol 18:369–380
Thway K, Rockcliffe S, Gonzalez D, Swansbury J, Min T, Thompson L, Fisher C (2010) Utility of sarcoma-specific fusion gene analysis in paraffin-embedded material for routine diagnosis at a specialist centre. J Clin Pathol 63:508–512
Trautmann M, Sievers E, Aretz S, Kindler D, Michels S, Friedrichs N, Renner M, Kirfel J et al (2014) SS18-SSX fusion protein-induced Wnt/β-catenin signaling is a therapeutic target in synovial sarcoma. Oncogene 33:5006–5016
Tsuda M, Watanabe T, Seki T, Kimura T, Sawa H, Minami A, Akagi T, Isobe KI, Nagashima K, Tanaka S (2005) Induction of p21 ( WAF1/CIP1) by human synovial sarcoma-associated chimeric oncoprotein SYT-SSX1. Oncogene 24:7984–7990
Van de Rijn M, Barr FG, Collins MH, Xiong QB, Fisher C (1999) Absence of SYT-SSX fusion products in soft tissue tumors other than synovial sarcoma. Am J Clin Pathol 112:43–49
Wang YJ, Wen SC, Chien ST, Sheu JW, Hsuea CW, Feng NH (2006) Primary intra-abdominal synovial sarcoma. J Chin Med Assoc 69:492–495
Wang ZH, Wang XC, Xue M (2010) Clinicopathologic analysis of 4 cases of primary renal synovial sarcoma. Chin J Cancer 29:212–216
Weidner N, Goldman R, Johnston J (1993) Epithelioid monophasic synovial sarcoma. Ultrastruct Pathol 17:287–294
Weiss SW, Goldblum JR (2001) Enzinger and Weiss’s soft tissue tumors, 4th edn. Mosby, St Louis, pp 1483–1509
Xiong B, Chen M, Ye F, Zhang Z, Yin L, Huang H, Chen H, Zhang H (2013) Primary monophasic synovial sarcoma of the liver in a 13-year-old boy. Pediatr Dev Pathol 16:353–356
Zajak J, Hladik P, Lischke R (2015) Synovial sarcoma of the liver – case report ( in Czech ). Rozhl Chir 94:78–81
Zenmyo M, Komiya S, Hamada T, Hiraoka K, Nagata K, Tsuji S, Hashimoto H, Inoue A (2001) Intraneural monophasic synovial sarcoma: a case report. Spine 26:310–313
Zhang Q, Wang H, Ren L, Qi X, Liu F, Zhang D (2014) Primary synovial sarcoma of the prostate metastatic to the liver and lung: a case report. World J Surg Oncol 12:194
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Zimmermann, A. (2017). Synovial-Like Neoplasms (Synovial Sarcoma) of the Liver. In: Tumors and Tumor-Like Lesions of the Hepatobiliary Tract. Springer, Cham. https://doi.org/10.1007/978-3-319-26956-6_70
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