Abstract

Background: Alveolar soft part sarcoma (ASPS) forms a rare group of sarcomas. ASPS is characterized by a relatively indolent clinical course, but the prognosis is eventually poor. In ophthalmic literature there is only a single case report on ASPS metastasis to the choroid. The aim of this report was to describe another patient with choroidal metastasis of ASPS.
Case Report: A 39-year-old woman was referred to the Clinic of Ophthalmology in Krakow, Poland. On examination in February 2009, a gray-yellow tumor located inferiorly in the left eye 6 mm from the edge of the optic disc was diagnosed. On B-scan ultrasound, the tumor base measured 14.7×15.6 mm and its apical thickness was 6.9–7.3 mm. MRI showed a tumor measuring 7×14×13 mm with elevated intensity on T1- and T2-weighted scans. In 1998 this patient had had surgical resection of a tumor in the left thigh. Histopathological examination revealed ASPS. In 2005, distant metastases to the lung were diagnosed and the patient underwent VAI chemotherapy. Because of poor response to chemotherapy, enucleation was recommended. The patient did not agree and I-125 brachytherapy was performed. Insufficient regression of the tumor associated with secondary exudative retinal detachment prompted the patient to decide to have the blind eye removed. The uneventful surgery took place in July 2009. Histopathology, PAS staining, and the immunohistochemical profile allowed us to diagnose ASPS metastasis to the choroid.
Conclusions: In very rare instances, the choroid may be the location of atypical metastases. ASPS metastasizes to the eye extremely rarely.

Keywords: alveolar soft-part sarcoma, intraocular tumor, Uvea, Choroid, metastasis