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- Title
Rare Occurrence of an Intraocular Choroidal Solitary Fibrous Tumor/Hemangiopericytoma.
- Authors
Rinaldo, Lorenzo; Xu, Sarah Chao Ying; Eggers, Scott D.; Salomão, Diva R.; Chen, John J.; Raghunathan, aditya
- Abstract
<bold><italic>Purpose:</italic></bold> Tumors previously diagnosed as solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) are characterized by the <italic>NAB2-STAT6</italic> fusion gene, leading to nuclear STAT6 expression, and are now considered part of one SFT/HPC tumor entity by the 2016 World Health Organization Classification of Tumors of the Central Nervous System. We present the first primary choroidal SFT/HPC with the diagnosis confirmed by STAT6 expression. <bold><italic>Procedures:</italic></bold> A 51-year-old man underwent enucleation for a choroidal mass, which revealed a spindle cell neoplasm involving the optic nerve, without extrascleral extension. Immunohistochemical stains for S-100, melan-A, tyrosinase, and HMB45 were all negative; however, detection of monosomy 3 by FISH favored a choroidal spindle cell melanoma. Four years later, he presented with hepatic metastases of a spindle cell tumor, and a year later with an epithelioid malignancy involving the calvarium. <bold><italic>Results:</italic></bold> The calvarial tumor showed nuclear STAT6 immunoreactivity, supporting the diagnosis of SFT/HPC. Retrospectively, the choroidal and hepatic masses were also found to demonstrate nuclear STAT6 expression, supporting the diagnosis of a primary choroidal SFT/HPC with metachronous metastases to the liver and calvarium. <bold><italic>Conclusions:</italic></bold> This case highlights the significance of considering SFT/HPC in the diagnosis of intraocular spindle cell tumors and the importance of STAT6 immunohistochemistry in the evaluation of such tumors.
- Publication
Ocular Oncology & Pathology, 2018, Vol 4, Issue 4, p213
- ISSN
2296-4681
- Publication type
Article
- DOI
10.1159/000481947