J Korean Ophthalmol Soc.  2012 May;53(5):716-720.

A Case of Primary Orbital Synovial Sarcoma

Affiliations
  • 1Department of Ophthalmology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea. eyeyang@inje.ac.kr

Abstract

PURPOSE
To report a rare case of primary orbital synovial sarcoma.
CASE SUMMARY
A 55-year-old male visited our clinic with a 2-month history of decreased visual field in the left eye. The best corrected visual acuity of the left eye was 1.0, and 2.5 mm of proptosis was observed. There was slight limitations in motility and a mild visual field defect in the inferotemporal quadrant of the left eye. Funduscopic examination of the left eye revealed papilledema. Orbital magnetic resonance imaging (MRI) demonstrated the presence of an orbital mass that extended inferotemporally into the left orbit. On magnetic resonance imaging, a well-demarcated and 2.7 x 2.0 x 2.2 cm sized tumor was identified with intermediate signal intensities on T1/T2-weighted scans with slight homogenous enhancement. We approached the orbital mass using a lateral conjunctival incision with left canthotomy and cantholysis and performed partial surgical resection of the mass because the mass encircled the optic nerve. The mass was diagnosed as synovial sarcoma based on the findings of both histopathology and immunohistochemical staining. Postoperatively, the patient underwent radiation therapy to the rest of the tumor site.
CONCLUSIONS
Synovial sarcoma should be considered in the differential diagnosis of an orbital mass lesion encircling the optic nerve. Radiation therapy is useful to treat completely unresectable primary synovial sarcoma.

Keyword

Orbital mass; Primary synovial sarcoma; Radiation therapy; Surgical resection

MeSH Terms

Diagnosis, Differential
Exophthalmos
Eye
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Optic Nerve
Orbit
Papilledema
Sarcoma, Synovial
Visual Acuity
Visual Fields

Figure

  • Figure 1 Magnetic Resonance Imaging of the left orbit demonstrates a large, homogenous, well-circumscribed mass in the retrobulbar space. The mass abuts onto the optic nerve and compresses it superomedially. Axial (A) and coronal (B) views.

  • Figure 2 Higher magnification of the biphasic synovial sarcoma specimen (hematoxylin & eosin stain [H&E]; original magnification, ×400). The spindle cell component is elongated cells with spindled hyperchromatic nuclei and scant amounts of cytoplasm. The epithelioid cell component shows cuboidal to round cells with oval to round nuclei, abundant cytoplasm, and prominent nucleoli.

  • Figure 3 Higher magnification of the biphasic synovial sarcoma specimen (immunohistochemical stain; original magnification, ×200). (A) Vimentin immunohistochemical staining of the tissue shows diffuse and dense positivity in the spindle cell component. (B) Epithelial membrane antigen (EMA) immunohistochemical staining highlights the epithelial component of this biphasic synovial sarcoma.

  • Figure 4 Magnetic Resonance Imaging of the left orbit shows known synovial sarcoma remnant. As compared with previous imaging, it was decreased more than 50% in volume (2.7 × 2.0 × 2.2 cm → 1.2 × 1.4 × 1.8 cm). Axial (A) and coronal (B) views.


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