Primary Orbital Synovial Sarcoma Mimicking a Periocular Cyst

Am J Dermatopathol. 2019 Sep;41(9):655-660. doi: 10.1097/DAD.0000000000001351.

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.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Biopsy, Needle
  • Diagnosis, Differential
  • Epidermal Cyst / diagnosis
  • Epidermal Cyst / pathology*
  • Epidermal Cyst / surgery
  • Humans
  • Immunohistochemistry
  • In Situ Hybridization, Fluorescence
  • Magnetic Resonance Imaging / methods
  • Orbit
  • Preoperative Care / methods
  • Prognosis
  • Rare Diseases
  • Risk Assessment
  • Sarcoma, Synovial / diagnosis
  • Sarcoma, Synovial / pathology*
  • Sarcoma, Synovial / surgery*
  • Skin Neoplasms / diagnosis
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / surgery*
  • Treatment Outcome
  • Ultrasonography / methods
  • Young Adult