Primary orbital monophasic synovial sarcoma with calcification: A case report

Abstract

BACKGROUND Synovial sarcoma is a malignant mesenchymal neoplasm with variable epithelial differentiation. Most synovial sarcoma cases are reported in young adults and can arise in any body site. Notably, primary orbital synovial sarcoma is rare. CASE SUMMARY An 8-year-old east Asian girl with 1-month history of gradual painless proptosis and lacrimation of the right eye was admitted. The patient presented with painless proptosis, downward eyeball displacement, and upward movement disorders. According to clinical manifestations, imaging examinations and postoperative immunohistochemical examinations, the diagnosis was monophasic synovial sarcoma with calcification. The patient underwent anterior orbitotomy procedure for removal of the right orbital mass under general anesthesia. The diagnosis of monophasic synovial sarcoma with calcification was confirmed finally through histological and immunohistochemical exam. The follow-up period was 6 mo, and no recurrence was observed during this period. CONCLUSION Primary orbital monophasic synovial sarcoma with calcification is a rare sarcoma, and clinical manifestations and imaging results are not specific. The tumor may present similar features as a benign tumor. Comprehensive analysis of clinical, radiological, and pathological findings is critically important for making the right diagnosis. Conventional treatment approach for synovial sarcoma is surgical resection with adjuvant or neoadjuvant radiotherapy, which is highly effective for localized tumors.

Commentary on "Primary orbital monophasic synovial sarcoma with calcification: A case report"

“…We read the article "Primary orbital monophasic synovial sarcoma with calcification: A case report"[ 1 ] with great interest and appreciated the authors for this comprehensive case report. We also thought that it might be favorable to contribute additional information about differential diagnosis and shortly immunohistochemical features to the discussion.…”

Ambivalent and Conjectural-Synovial Sarcoma

“…Synovial sarcoma is immune reactive to TLE1 and various cytokeratin wherein epithelial component is consistently immune reactive to CK7, CK8, CK14, CK18, CK19 and variably reactive to CK17, CK13, CK6 or CK16. Spindle cell component is focally and variably immune reactive to CK7, CK19, CK18, CK8, CK14, CK17 or CK20 [3,4]. Synovial sarcoma is immune reactive to epithelial membrane antigen (EMA), BCL2, β-catenin, calponin, CD99, CD56, CD57 or calretinin [3,4].…”

“…Spindle cell component is focally and variably immune reactive to CK7, CK19, CK18, CK8, CK14, CK17 or CK20 [3,4]. Synovial sarcoma is immune reactive to epithelial membrane antigen (EMA), BCL2, β-catenin, calponin, CD99, CD56, CD57 or calretinin [3,4]. Epithelial component is comprehensively (100%) immune reactive to keratin or epithelial membrane antigen (EMA) whereas spindle cell component is immune reactive in ~80% instances [3,4].…”

“…Synovial sarcoma is immune reactive to epithelial membrane antigen (EMA), BCL2, β-catenin, calponin, CD99, CD56, CD57 or calretinin [3,4]. Epithelial component is comprehensively (100%) immune reactive to keratin or epithelial membrane antigen (EMA) whereas spindle cell component is immune reactive in ~80% instances [3,4]. Besides, neoplasm is immune reactive to SS18-SSX fusion specific antibody, SSX C terminus antibody or NY-ESO-1 [3,4].…”

“…Epithelial component is comprehensively (100%) immune reactive to keratin or epithelial membrane antigen (EMA) whereas spindle cell component is immune reactive in ~80% instances [3,4]. Besides, neoplasm is immune reactive to SS18-SSX fusion specific antibody, SSX C terminus antibody or NY-ESO-1 [3,4]. Synovial sarcoma is immune non-reactive to CD34, desmin, h-caldesmon, myogenin, MyoD1, FLI1, WT1, SOX10, S100 protein or H3K27me3 [3,4].…”