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.

Primary Orbital Synovial Sarcoma Mimicking a Periocular Cyst / Portelli, Francesca; Pieretti, Giulia; Santoro, Nicola; Gorelli, Greta; De Giorgi, Vincenzo; Massi, Daniela; Dei Tos, Angelo Paolo; Mazzini, Cinzia. - In: AMERICAN JOURNAL OF DERMATOPATHOLOGY. - ISSN 0193-1091. - ELETTRONICO. - (2019), pp. 1-6. [10.1097/DAD.0000000000001351]

Primary Orbital Synovial Sarcoma Mimicking a Periocular Cyst

Portelli, Francesca;Pieretti, Giulia;Santoro, Nicola;Gorelli, Greta;De Giorgi, Vincenzo;Massi, Daniela;Mazzini, Cinzia
2019

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.
2019
1
6
Portelli, Francesca; Pieretti, Giulia; Santoro, Nicola; Gorelli, Greta; De Giorgi, Vincenzo; Massi, Daniela; Dei Tos, Angelo Paolo; Mazzini, Cinzia...espandi
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1160663
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