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Primary superficial synovial sarcoma: clinical and histopathological characteristics in eight cases with molecular confirmation
Ist Teil von
Journal of cutaneous pathology, 2021-02, Vol.48 (2), p.263-268
Ort / Verlag
Oxford, UK: Blackwell Publishing Ltd
Erscheinungsjahr
2021
Link zum Volltext
Quelle
Wiley Online Library - AutoHoldings Journals
Beschreibungen/Notizen
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.