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.
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|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.|