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Diffuse Histiocytic Sarcoma Involving the Choroid ONLINE FIRST

Yamini Krishna, MBChB, PhD, FRCOphth1; Iguaracyra Araujo, MD, PhD1,2; Sunil Warrier, MBBS, MMed, FRANZCO3,4; Heinrich Heimann, MD, FRCOphth3; Sarah E. Coupland, MBBS, PhD, FRCPath1,5
[+] Author Affiliations
1Department of Cellular Pathology, Royal Liverpool and Broadgreen University Hospital Trust, Liverpool, England
2Department of Pathology and Forensic Medicine, Faculty of Medicine–Federal University of Bahia, Salvador, Brazil
3Liverpool Ocular Oncology Centre, Royal Liverpool and Broadgreen University Hospital Trust, Liverpool, England
4Queensland Ocular Oncology Service, Terrace Eye Centre, Brisbane, Australia
5Department Molecular and Clinical Cancer Medicine, University of Liverpool, Liverpool, England
JAMA Ophthalmol. Published online August 18, 2016. doi:10.1001/jamaophthalmol.2016.2867
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This case report describes a patient with systemic histiocytic sarcoma and choroidal infiltration of sarcoma cells.

Herein, we describe a rare case of histiocytic sarcoma with choroidal involvement with immunohistochemical and genetic confirmation. To our knowledge, this is the first report of histiocytic sarcoma with intraocular involvement in the human eye.

Article InformationCorresponding Author: Sarah E. Coupland, MBBS, PhD, FRCPath, Department Molecular and Clinical Cancer Medicine, University of Liverpool, Apex Bldg, 6 W Derby St, Liverpool L7 8TX, England (s.e.coupland@liverpool.ac.uk).

Published Online: August 18, 2016. doi:10.1001/jamaophthalmol.2016.2867.

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Additional Contributions: We thank Geetha Menon, MBBS, FRCPath, and Stuart Ruthven, MBBS, FRCPath, of the Hemato-Oncology Diagnostic Service at the Royal Liverpool University Hospital for their initial workup of the presenting lymph node in the patient. No compensation was received.

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Optical Coherence Tomographic (OCT) Findings and Immunohistochemistry Results

Optical coherence tomographic scans of the right (A) and left eye (B). C, Histiocytic sarcoma cells infiltrate the choroid (Ch), but the sclera (S), retina (R), and optical nerve (ON) were tumor free (hematoxylin-eosin, original magnification ×4). D, Histiocytic sarcoma cells infiltrate the choroid (hematoxylin-eosin, original magnification ×20). E, Neoplastic cells showed prominent/convoluted nuclei in choroidal blood vessels (arrows) (hematoxylin-eosin, original magnification ×40). F, Histiocytic sarcoma cells are CD163+ cells (3,3’-diaminobenzidine, original magnification ×25). G, CD68+ cells. H, Histiocytic sarcoma cells were negative for Langerhans cell (CD1a and Langerin), follicular dendritic cell (CD21 and CD35), myeloid cell (myeloperoxidase, CD33, and CD13), and lymphoid cell markers (CD3, CD20, CD79a, and PAX5) and Melan-A (note the choroidal melanocytes between the negative tumor cells).

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