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Shields, Carol L. MD*; Roe, Richard MD, MHS; Yannuzzi, Lawrence A. MD; Shields, Jerry A. MD*

Retinal Cases & Brief Reports: January 2017 - Volume 11 - Issue 1 - p 18–23
doi: 10.1097/ICB.0000000000000278
Case Report

Purpose: To report novel observations of previously described solitary circumscribed retinal astrocytic proliferation using spectral domain optical coherence tomography that suggests this tumor does not arise in the nerve fiber layer as initially believed, but arises within deep retinal or retinal pigment epithelial structures.

Methods: Retrospective review of four cases.

Results: Patient age ranged from 46 to 75 years. The tumor was pearl white or yellow-white (n = 4, 100%), located in the macula (n = 1, 25%) or macula to equator (n = 3, 75%) regions, and with mean tumor base of 1.2 mm and thickness of 0.8 mm. There were no feeding vessels, intrinsic vessels, subretinal fluid, or vitreoretinal traction. Mild surrounding retinal pigment epithelial hyperplasia and atrophy rimmed each tumor (n = 4, 100%). Fluorescein angiography depicted the mass with early hypofluorescence (n = 3/3, 100%) and late hypofluorescence (n = 2/3, 67%). Spectral domain optical coherence tomography demonstrated the mass with an abruptly elevated “snowball” configuration (n = 4, 100%), with smooth or slightly irregular surface (n = 4, 100%), and originating from deep retina or retinal pigment epithelial (n = 4, 100%), with overlying compression and draping of retinal tissue (n = 4, 100%).

Conclusion: This previously described small yellow-white retinal tumor appears to arise in the outer retinal layers and not from the inner retinal layers as formerly believed. This tumor may not be astrocytic as initially believed since it arises deep within the retina, but it could represent a deep glial or pigment epithelial fibrous mass. The pathogenesis and pathology of this rare lesion remain unknown.

Four cases of solitary circumscribed retinal astrocytic proliferation were evaluated with multimodal imaging and this avascular “pearl white” lesion appears to arise in the deep retina or retinal pigment epithelium and not in the nerve fiber layer, as originally suspected.

*Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, Pennsylvania;

Retina-Vitreous Associates Medical Group, Los Angeles, California; and

Retina-Vitreous, Macula Associates, New York City, New York.

Reprint requests: Carol L. Shields, MD, Ocular Oncology Service, Suite 1440, Wills Eye Institute, 840 Walnut Street, Philadelphia, PA 19107; e-mail:

Supported by the Eye Tumor Research Foundation, Philadelphia, PA (C.L.S., J.A.S.). The funders had no role in the design and conduct of the study, in the collection, analysis, and interpretation of the data, and in the preparation, review, or approval of the manuscript. C. L. Shields, MD has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

None of the authors have any conflicting interests to disclose.

© 2017 by Ophthalmic Communications Society, Inc.