Advertisement

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

Published:September 24, 2020DOI:https://doi.org/10.1016/j.oooo.2020.08.016
      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.
      To read this article in full you will need to make a payment

      References

        • 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