Vol 4 | Issue 2 | July-Dec 2018 | Page 14-19 | Kala Gnanasekaran Kiruthiga, Anne Jennifer Prabhu, Rekha Pai, Sramana Mukhopadhyay, Reka.K, V.T.K.Titus, Selvamani Backianathan.
Authors: Kala Gnanasekaran Kiruthiga , Anne Jennifer Prabhu , Rekha Pai , Sramana Mukhopadhyay , Reka.K , V.T.K.Titus , Selvamani Backianathan .
 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India.
 Department of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India.
 Department of Orthopaedics, Christian Medical College, Vellore, Tamil Nadu, India.
 Department of Radiotherapy, Christian Medical College, Vellore, Tamil Nadu, India.
Address of Correspondence
Dr. Anne Jennifer Prabhu,
Address: Department of Pathology, ASHA Building, Christian Medical College, Vellore, Tamil Nadu, India.
Context: Synovial sarcoma is one of the commonly encountered spindle cell sarcomas of soft tissue. However, Biphasic synovial sarcoma (BSS) is a rare subtype of synovial sarcoma with limited literature on clinical profile, molecular characteristics and survival outcome.
Aims: We propose to describe the immuno – morphology, clinical features, molecular profile and outcome of patients with BSS.
Settings and Design: This retrospective study included 13 cases of BSS, 3.2% of all synovial sarcomas diagnosed over 10 years in our institute. The clinico-pathological features were studied in detail and immunohistochemistry for TLE-1, EMA, CD34, CD99 and S100 was done. Real time PCR and DNA sequencing for the common translocations (SYT-SSX1/ SYT-SSX2) were performed.
Statistical analysis used: Statistical Package for Social Services (SPSS) software Version 21.0 (Armonk, NY: IBM Corp).
Results: BSS was most commonly seen in young, the most common site being soft tissue of extremities (92.3%). 53.8% of patients presented at Enneking stage IIB. The FNCLCC grade varied between 2(46.2%) and 3(53.8%). All cases were positive for EMA and TLE-1; negative for CD34 and S100. Ten of the eleven (90.9%) patients tested had SYT-SSX1 translocation. Over a period of 8 to 46 months, 53.8% cases were alive and well with no evidence of disease; three had (30%) recurred, one (10%) had lung metastasis and one (10%) died.
Conclusions: BSS is most common in extremities. The immunohistochemical profile matches that of monophasic synovial sarcoma. FNCLCC grade is 2 to 3; however the grade does not correlate with clinical outcome. Most cases show SYT-SSX1 translocation. 53.8% cases were alive and well after a mean follow up of 20 months.
Keywords: Biphasic, follow-up, immunoprofile, SYT-SSX1, synovial sarcoma.
Keyword: Biphasic synovial sarcoma has the same immunoprofile as the monophasic subtype. All patients who tested positive had SYT-SSX1 translocation. More than 50% of patients were alive and well after 20 months.
1. Herzog CE. Overview of sarcomas in the adolescent and young adult population. J Pediatr Hematol Oncol 2005;27:215–8.
2. Suurmeijer AJH, de Bruijn D, Geurts van Kessel A, Miettinen MM. Tumours of uncertain differentiation. In: Fletcher C.D.M, Bridge JA, Hogendoorn P.C.W, Mertens F, editors. WHO Classification of Tumours of Soft Tissue and Bone, 4th edn. IARC: Lyon; 2013.p. 213-5.
3. Terry J, Saito T, Subramanian S, Ruttan C, Antonescu CR, Goldblum JR et al. TLE1 as a diagnostic immunohistochemical marker for synovial sarcoma emerging from gene expression profiling studies. Am J Surg Pathol 2007;31:240–6.
4. Christopher D.M.Fletcher. Tumours of soft tissue. In: Fletcher. C.D.M, editor. Diagnostic Histopathology of Tumors, 4th edn. Philadelphia: Saunders – Elsevier; 2013.p. 1847-9.
5. Goldblum JR, Folpe AL, Enzinger FM, Weiss SW. Malignant Soft Tumours of Uncertain Type. In: Enzinger and Weiss’s soft tissue tumors, 6th edn. Philadelphia: Saunders – Elsevier; 2014.p. 1052 – 70.
6. Coindre JM, Trojani M, Contesso G, David M, Rouesse J, Bui NB et al. Reproducibility of a histopathologic grading system for adult soft tissue sarcoma. Cancer 1986;58:306–9.
7. Guillou L, Benhattar J, Bonichon F, Gallagher G, Terrier P, Stauffer E et al. 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 2004; 22: 4040-50.
8. Krieg AH, Hefti F, Speth BM, Jundt G, Guillou L, Exner UG 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;22:458-67.
9. Trojani M, Contesso G, Coindre JM, Rouesse J, Bui NB, de Mascarel A et al. Soft-tissue sarcomas of adults – study of pathological prognostic variables and definition of a histopathological grading system. Int J Cancer 1984;33:37–42.
10. Ferrari A, De Salvo GL, Brennan B, van Noesel MM, De Paoli A, Casanova M et al. Synovial sarcoma in children and adolescents: the European pediatric Soft tissue sarcoma Study Group prospective trial (EpSSG NRSTS 2005) Ann Oncol 2015;26 :567–72.
11. Ladanyi M, Antonescu CR, Leung DH, Woodruff JM, Kawai A, Healey JH et al. Impact of SYT-SSX fusion type on the clinical behavior of synovial sarcoma: a multi-institutional retrospective study of 243 patients. Cancer Res 2002;62:135–40.
12. Mallen-St Clair J, Arshi A, Abemayor E, St John M. Factors Associated With Survival in Patients With Synovial Cell Sarcoma of the Head and Neck: An Analysis of 167 Cases Using the SEER (Surveillance, Epidemiology, and End Results) Database. JAMA Otolaryngol– Head Neck Surg 2016;142:576–83.
13. Osma U, Eyigor H, Suren D, Sezer C, Yilmaz MD. Biphasic synovial sarcoma of the hypopharynx. Ear Nose Throat J 2015;94:36–9.
14. Sharif MA, Mushtaq S, Mamoon N, Khadim MT, Asghar Z. Biphasic synovial sarcoma of oral cavity. J Coll Physicians Surg–Pak JCPSP 2008;18:713–5.
15. Hazelbag HM, Szuhai K, Tanke HJ, Rosenberg C, Hogendoorn PCW. Primary synovial sarcoma of the heart: a cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype. Mod Pathol Off J U S Can Acad Pathol Inc 2004;17:1434–9.
16. Jun L, Ke S, Zhaoming W, Linjie X, Xinru Y. Primary synovial sarcoma of the prostate: report of 2 cases and literature review. Int J Surg Pathol 2008;16:329–34.
17. Makhlouf HR, Ahrens W, Agarwal B, Dow N, Marshalleck JJ, Lee EL et al. Synovial sarcoma of the stomach: a clinicopathologic, immunohistochemical, and molecular genetic study of 10 cases. Am J Surg Pathol 2008;32:275–81.
18. Vera J, García M-D, Marigil M, Abascal M, Lopez J-I, Ligorred L. Biphasic synovial sarcoma of the abdominal wall. Virchows Arch Int J Pathol 2006;449:367–72.
19. Guillou L, Wadden C, Kraus MD, Tos APD, Fletcher CDM. S-100 Protein Reactivity in Synovial Sarcomas—a Potentially Frequent Diagnostic Pitfall. Appl Immunohistochem 1996;4:167–75.
20. Olsen SH, Thomas DG, Lucas DR. Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma. Mod Pathol 2006;19:659-68.
21. Hui P, Li N, Johnson C, De Wever I, Sciot R, Manfioletti G et al. HMGA proteins in malignant peripheral nerve sheath tumor and synovial sarcoma: preferential expression of HMGA2 in malignant peripheral nerve sheath tumor. Mod Pathol 2005;18:1519-26.
22. Cleven AH, Sannaa GA, Briaire-de Bruijn I, Ingram DR, van de Rijn M, Rubin BP et al. Loss of H3K27 tri-methylation is a diagnostic marker for malignant peripheral nerve sheath tumors and an indicator for an inferior survival. Mod Pathol 2016 J;29:582-90.
23. Pelmus M, Guillou L, Hostein I, Sierankowski G, Lussan C, Coindre J-M. Monophasic fibrous and poorly differentiated synovial sarcoma: immunohistochemical reassessment of 60 t(X;18)(SYT-SSX)- positive cases. Am J Surg Pathol 2002;26:1434–40.
24. Dei Tos AP, Calonje E, Sciot R, Pauwels P, Knight JC, Dal Cin P et al. Immunohistochemical demonstration of glycoprotein p30/32MIC2 (CD99) in synovial sarcoma. A potential cause of diagnostic confusion. App Immunhistochem 1995;3:168-73.
|How to Cite this article: Kiruthiga KG, Anne Jennifer P, Rekha P, Sramana M, Reka.K, V.T.K.Titus, Selvamani B.. Biphasic synovial sarcoma – A ten year experience with molecular profile and clinical outcome. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 14-19.