Biphasic synovial sarcoma – A ten year experience with molecular profile and clinical outcome
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 [1], Anne Jennifer Prabhu [1], Rekha Pai [1], Sramana Mukhopadhyay [1], Reka.K [2], V.T.K.Titus [3], Selvamani Backianathan [4].
[1] Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India.
[2] Department of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India.
[3] Department of Orthopaedics, Christian Medical College, Vellore, Tamil Nadu, India.
[4] 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.
E-mail: annejennifer91@gmail.com
Abstract
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. |
