Calcifying synovial sarcoma of the tongue with SS18 rearrangement: a rare variant in a rare location

Published:September 24, 2020DOI:
      Synovial sarcoma is a soft tissue malignancy harboring t(X;18) resulting in fusion of two genes SS8 (at 18q11) and SSX (1, 2 or 4 at Xp11) forming the gene fusion product SS18-SSX. It affects adults in their 3rd-4th decades, most frequently in the para-articular regions of the extremities. Less than 10% of the cases occur within the head and neck region and of these, 60% occur in the neck and only 10% occur in the oral cavity. We report a synovial sarcoma of the tongue in a 14-year-old female patient with unusual histology. The patient presented with a mass occupying most of the tongue with extension into the floor of mouth and the lingual gingiva of the anterior mandibular teeth. The tumor was composed of a highly cellular proliferation of spindle cells in a herringbone pattern with many small vessels but without glandular structures, and with extensive calcifications throughout the tumor. Tumor cells were positive for epithelial membrane antigen and transducin-like enhancer of split-1, and fluorescence in situ hybridization studies identified SS18 gene rearrangement. The patient was managed with two debulking procedures followed by chemoradiation and is currently alive with disease.
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        • Varela-Duran J
        • Enzinger F
        Calcifying synovial sarcoma.
        Cancer. 1982; 50: 345-352
        • Fisher C.
        Ultrastructural and immunohistochemical features of epithelial differentiation in monophasic and biphasic tumors.
        Hum Pathol. 1986; 17: 996-1008
        • Skytting B
        • Nilsson G
        • Brodin B
        • et al.
        A novel fusion gene, SYT-SSX4, in synovial sarcoma.
        J Natl Cancer Inst. 1999; 91: 974-975
        • Ladanyi M.
        Fusions of the SYT and SSX genes in synovial sarcoma.
        Oncogene. 2001; 20: 5755-5762
        • Bianchi G
        • Sambri A
        • Righi A
        • Dei Tos A
        • Picci P
        • Donati D
        Histology and grading are important prognostic factors in synovial sarcoma.
        Eur J Surg Oncol. 2017; 43: 1733-1739
        • Sultan I
        • Rodriguez-Galindo C
        • Saab R
        • Yasir S
        • Casanova M
        • Ferrari A
        Comparing children and adults with synovial sarcoma in the Surveillance, Epidemiology, and End Results program, 1983 to 2005: an analysis of 1268 patients.
        Cancer. 2009; 115: 3537-3547
        • Miettinen M
        Virtanen I. Synovial sarcoma—a misnomer.
        Am J Pathol. 1984; 117: 18
        • Corey RM
        • Swett K
        • Ward WG
        Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database report.
        Cancer Med. 2014; 3: 1404-1415
        • Harb WJ
        • Luna MA
        • Patel SR
        • Ballo MT
        • Roberts DB
        • Sturgis EM
        Survival in patients with synovial sarcoma of the head and neck: association with tumor location, size, and extension.
        Head Neck. 2007; 29: 731-740
        • Owosho AA
        • Estilo CL
        • Rosen EB
        • Yom SK
        • Huryn JM
        • Antonescu CR
        A clinicopathologic study on SS18 fusion positive head and neck synovial sarcomas.
        Oral Oncol. 2017; 66: 46-51
        • Novotny G
        • Fort T.
        Synovial sarcoma of the tongue.
        Arch Otolaryngol. 1971; 94: 77-79
        • Shmookler B
        • Enzinger U
        • Brannon R
        Orofacial synovial sarcoma: a clinicopathologic study of 11 new cases and review of the literature.
        Cancer. 1982; 50: 269-276
        • Engelhardt S
        • Leafstedt S.
        Synovial sarcoma of tonsil and tongue base.
        South Med J. 1983; 76: 243-244
        • Holtz F
        • Magielski J.
        Synovial sarcomas of the tongue base: the seventh reported case.
        Arch Otolaryngol. 1985; 111: 271-272
        • Bridge J
        • Bridge R
        • Borek D
        • Shaffer B
        • Norris C
        Translocation t(X;18) in orofacial synovial sarcoma.
        Cancer. 1988; 62: 935-937
        • Carrillo R
        • El-Naggar A
        • Rodriguez-Peralto J
        • Batsakis J
        Synovial sarcoma of the tongue: case report and review of the literature.
        J Oral Maxillofac Surg. 1992; 50: 904-906
        • Komis C
        • Lagogiannis GA
        • Faratzis G
        • Rapidis AD
        Synovial sarcoma of the tongue: report of a case and review of the literature.
        J Oral Maxillofac Surg. 2008; 66: 154-160
        • Agarwal A
        • Shet T
        • Joshi R
        • Desai S
        • Chinoy R
        Monophasic synovial sarcoma of tongue.
        Indian J Pathol Microbiol. 2009; 52: 568-570
        • de Almeida-Lawall M
        • Mosqueda-Taylor A
        • Bologna-Molina RE
        • et al.
        Synovial sarcoma of the tongue: case report and review of the literature.
        J Oral Maxillofac Surg. 2009; 67: 914-920
        • Villaroel-Salinas J
        • Campos-Martinez J
        • Ortiz-Hidalgo C
        Synovial sarcoma of the tongue confirmed by molecular detection of the SYT-SSX2 fusion gene transcript.
        Int J Surg Pathol. 2012; 20: 386-389
        • Basile L
        • Hoch B
        • Dillon J
        Synovial sarcoma of the tongue: report of a case.
        J Oral Maxillofac Surg. 2016; 74: 95-103
        • Su Z
        • Zhang J
        • Gao P
        • et al.
        Synovial sarcoma of the tongue: report of a case and review of the literature.
        Ann R Coll Surg Engl. 2018; 100 (e118-e122)
        • Moussavi H
        • Ghodsi S.
        Synovial sarcoma of the tongue: report of a case.
        J Laryngol Otol. 1974; 88: 795-797
        • de Silva M
        • McMahon A
        • Paterson L
        • Reid R
        Identification of poorly differentiated synovial sarcoma: a comparison of clinicopathological and cytogenetic features with those of typical synovial sarcoma.
        Histopathology. 2003; 43: 220-230
        • Wilkerson BW
        • Crim JR
        • Hung M
        • Layfield LJ
        Characterization of synovial sarcoma calcification.
        AJR Am J Roentgenol. 2012; 199: W730-W734
        • Jiang D
        • Peng R
        • Yan X
        • et al.
        Synovial sarcoma showing loss of a green signal in SS18 fluorescence in situ hybridization: a clinicopathological and molecular study of 12 cases.
        Virchows Arch. 2017; 471: 799-807
        • Ito J
        • Asano N
        • Kawai A
        • Yoshida A
        The diagnostic utility of reduced immunohistochemical expression of SMARCB1 in synovial sarcomas: a validation study.
        Hum Pathol. 2016; 47: 32-37
        • Foo WC
        • Cruise MW
        • Wick MR
        • Hornick JL
        Immunohistochemical staining for TLE1 distinguishes synovial sarcoma from histologic mimics.
        Am J Clin Pathol. 2011; 135: 839-844
        • Kosemehmetoglu K
        • Vrana JA
        • Folpe AL
        TLE1 expression is not specific for synovial sarcoma: a whole section study of 163 soft tissue and bone neoplasms.
        Mod Pathol. 2009; 22: 872-878
        • Baranov E
        • McBride M
        • Bellizzi A
        • et al.
        A novel SS18-SSX fusion-specific antibody for the diagnosis of synovial sarcoma.
        Am J Surg Pathol. 2020; 44: 922-933
        • Schaefer IM
        • Fletcher CD
        • Hornick JL
        Loss of H3 K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics.
        Mod Pathol. 2016; 29: 4-13
        • Doyle LA
        • Vivero M
        • Fletcher CD
        • Mertens F
        • Hornick JL
        Nuclear expression of STAT6 distinguishes solitary fibrous tumor from histologic mimics.
        Mod Pathol. 2014; 27: 390-395
        • Nielsen TO
        • Poulin NM
        • Ladanyi M
        Synovial sarcoma: recent discoveries as a roadmap to new avenues for therapy.
        Cancer Discov. 2015; 5: 124-134
        • Krieg AH
        • Hefti F
        • Speth BM
        • et al.
        Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors.
        Ann Oncol. 2011; 22: 458-467