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.
The results showed that visual acuity decreased significantly after a long follow-up period mainly because of the development of chorioretinal atrophy.
Introduction: We report about a large retinal capillary hemangioma (RCH) with exudative retinal detachment and a macular fold, treated with Ruthenium-106 brachytherapy (Ru-106 BT) and scleral buckling surgery, followed by pars plana vitrectomy (PPV), for the removal of macular tractions. Case description: A 17-year-old boy was referred to our Ocular Oncology Unit for a large RCH in the left eye. BCVA was hand motion. The RCH measured 4.9 × 6.85 mm in basal diameters and 4.0 mm in thickness and was located in the mid-peripheral temporal retina. It was surrounded by extensive subretinal exudation, forming an exudative retinal detachment, with a retinal fold that extended from the lesion to the optic disc. We performed Ru-106 BT and at the moment of the plaque removal we placed a radial buckle with the aim to unbend the retinal fold. At 3-months follow-up the exudation decreased, we achieved the opening of the peripheral side of the retinal fold, but the macula was still detached. We decided to perform a lens sparing PPV, macular peeling and air tamponade, to remove the vitreoretinal tractions ab interno and to try to complete the opening of the macular fold. After 1-month BCVA was counting fingers, the retina appeared attached, also in the macular area, but the retinal fold remained partially close in the macular side. After 6 months the tumor was inactivated, the macula remained attached, unfortunately, the macular fold remained partially close. Conclusion: Ru-106 BT and scleral buckling concurrent approach can be an effective treatment modality in selected cases of large RCHs, followed by PPV to remove eventual vitreo-retinal tractions.
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