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Primary Orbital Synovial Sarcoma Mimicking a Periocular Cyst

Portelli, Francesca MD*; Pieretti, Giulia MD, PhD; Santoro, Nicola MD, PhD; Gorelli, Greta BSc*; De Giorgi, Vincenzo MD; Massi, Daniela MD, PhD*; Dei Tos, Angelo Paolo MD§; Mazzini, Cinzia MD

The American Journal of Dermatopathology: September 2019 - Volume 41 - Issue 9 - p 655–660
doi: 10.1097/DAD.0000000000001351
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Abstract: Synovial sarcoma (SS) is a high-grade soft-tissue sarcoma occurring predominantly in older children and young adults. Only approximately 7% occur in the head and neck region, with SS representing less than 0.1% of all head and neck cancers. Orbital location is exceedingly rare with only 8 cases reported so far in the literature. It is noted for its propensity for late local recurrences and metastases. Histologically, SS is monophasic, biphasic, or poorly differentiated and harbors a specific chromosomal translocation t(X;18)(p11.2;q11.2) in >95% of cases. In this article, we describe a case of monophasic SS primarily arising in the left supero-nasal orbital region in a 24-year-old woman, clinically mistaken for a periocular cyst. The case is peculiar for its highly unusual location and for its clinical deceptively benign appearance.

*Histopathology and Molecular Diagnostics, Department of Health Sciences, Careggi University Hospital, Florence, Italy;

Unit of Ocular Oncology, Department of Surgery and Translational Medicine, Careggi University Hospital, Florence, Italy;

Division of Dermatology, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy; and

§Department of Medicine, University of Padua School of Medicine, Padua, Italy.

Correspondence: Francesca Portelli, MD, Histopathology and Molecular Diagnostics, Department of Health Sciences, Careggi University Hospital, Largo Brambilla, 3, 50134 Florence, Italy (e-mail: portellif@aou-careggi.toscana.it).

The authors declare no conflicts of interest.

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