Primary synovial sarcoma of the orbit: a clinicopathological review
Journal Title: Eye Science (Yanke Xuebao) - Year 2021, Vol 36, Issue 8
Abstract
[Objective:] To investigate the clinicopathological and molecular genetics features of synovial sarcoma (SS) of the orbit. [Methods:] We retrospectively reviewed 10 published cases of primary SS of the orbit, along with 1 case of primary SS of the orbit confirmed by pathology who was admitted to the ophthalmology department of Eye & ENT Hospital of Fudan University in October 2020. The clinical data, radiological findings, morphology, immunophenotype and genetic characteristics of the cases were analyzed. [Results:] Our case was a 53-year-old woman with an SS in the right orbit, which had recurred multiple times. Histopathologic examination showed a primitive tumor composed of spindled and ovoid cells. Focal infiltration was observed in adjacent structures, such as the sub-conjunctiva, scleral surface, optic nerve sheath, muscle, and fibro-fatty tissue. Immunohistochemistry showed positivity for vimentin, calponin, CD99, and Bcl-2 and loss of INI-1expression, which is typical of SS. Fluorescence in situ hybridization (FISH) showed the (X;18) translocation in the tumor cells. The analysis included 2 males and 9 females aged between 1 and 53 years old (mean: 22 years; median: 24 years). Among the SS cases, 6 left eyes and 5 right eyes (all monocular cases) were affected. Symptoms had been present from 1 week to 13 years in the case from our hospital, while in 5 cases, symptoms had been present for at least 3 years. Common clinical features of the patients included proptosis or globe displacement, decreased vision, and pain. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an ovoid mass with heterogenous enhancement and a cystic appearance, which was probably attributable to hemorrhage or necrosis. Of these 11 cases, 7 cases were biphasic SS, 4 were monophasic fibrous SS, and 2 were poorly differentiated in monophasic SS. Immunohistochemistry revealed positivity for pan-cytokeratin (CKpan), CK7, CK19, vimentin, cluster of differentiation 99 (CD99), B-cell lymphoma 2 (Bcl-2), calponin and transducin-like enhancer protein 1 (TLE1). [Conclusion:] Primary SS of the orbit is extremely rare and needs to be distinguished from other spindle cell tumors of orbital soft tissue. The SS diagnosis is based on the presence of the t(X;18) (p11; q11) translocation, which results in an SYT-SSX fusion gene.
Authors and Affiliations
Hui SUN, Min YE, Rongrong CAI, Ji SUN, Yingwen BI
Keywords
Related Articles
- EP ID EP763546
- DOI 10.3978/j.issn.1000-4432.2021.05.04
- Views 17
- Downloads 0
Exploration and practice of the visual health management mode based on artificial intelligence-assisted diagnosis in Minhang District
[Objective] To explore and practice the visual health management mode of Minhang District. [Methods] Introduce the visual health management mode of AI-assisted diagnosis in Minhang District; analyze and compare the tradi...
Clinical distribution characteristics of patients admitted to hospital for surgical cataract treatment
[Objective:] To understand the clinical distribution characteristics of cataract surgery patients and provide reference for cataract prevention and treatment in primary hospitals. [Methods:] From January 2019 to December...
Nursing experience of 24,180 children undergoing ophthalmic surgery under general anesthesia with laryngeal mask during anesthesia recovery period
[Objective:] To explore holistic nursing strategies to improve the quality and efficiency of post-anesthesia care unit(PACU) resuscitation in childrenpatients undergoing ophthalmic laryngeal mask anesthesia. [Methods:] A...
Computed tomography and pathological manifestations of space-occupying lesions of the orbital bone and their relevance
[Objective:] To analyze computed tomography (CT) manifestations of space-occupying lesions of the orbital bone and their relationship with histopathology. [Methods:] The clinical data of 15 patients with orbital bone occ...
Association of myopia with the macular ganglion cell layer and inner plexiform layer in diabetic patients: A longitudinal study
[Objective:] To investigate the association between myopia and ganglion cell layer and inner plexiform layer (GCIPL) in diabetic population without diabetic retinopathy (DR). [Methods:] In this Guangzhou Diabetic Eye stu...