Abstract
Background and Aims: Synovial sarcoma (SS) is a spindled cell sarcoma demonstrating varying degrees of epithelial differentiation and characterized by a pathognomonic t(X;18) translocation. SS most frequently involves deep soft tissue of the extremities in young adults. Superficial SS involving dermis and/or subcutaneous tissue is exceedingly rare. Methods and results: We identified eight cases of primary superficial synovial sarcomas across three tertiary institutions. All cases were confined to the dermis/subcutis based on imaging or gross and microscopic examination. The average patient age was 36 years (range 14-50). The average tumor size was 2.4 cm (range 0.9-3.9 cm) and lesions showed classic monophasic (n = 4) or biphasic (n = 4) morphology. All tumors expressed keratin AE1/AE3 and/or epithelial membrane antigen (EMA), but were negative for CD34. The diagnosis for each case was confirmed by molecular detection of t(X;18). Six of the eight cases were treated with curative excision while the other two received additional radiotherapy. Follow-up was available for six patients (mean 68 months, range 2-108 months) and no patient experienced recurrence or metastatic disease. Conclusions: We present the largest series to date of primary superficial SS with molecular confirmation for all cases. SS should be considered when evaluating a cutaneous monomorphic spindle cell neoplasm.
Original language | English (US) |
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Pages (from-to) | 263-268 |
Number of pages | 6 |
Journal | Journal of cutaneous pathology |
Volume | 48 |
Issue number | 2 |
DOIs | |
State | Published - Feb 2021 |
Keywords
- cutaneous spindle cell sarcoma
- subcutaneous synovial sarcoma
- superficial synovial sarcoma
- t (X;18)
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Histology
- Dermatology