Presumed Solitary Circumscribed Retinal Astrocytic Proliferation

Abstract

Objective: To report the clinical features and differential diagnosis of an unusual entity termed presumed solitary circumscribed retinal astrocytic proliferation (PSCRAP). Methods: Retrospective review of medical records. Results: All patients with PSCRAP were asymptomatic, and the lesion was found during routine examination. There were 5 men and 2 women with a median age of 53 years. No patient had a history or clinical findings of tuberous sclerosis complex. Each PSCRAP lesion was circumscribed, abruptly elevated, and opaque white to yellow and mostly obscured the underlying retinal vessels. The lesions had no associated subretinal fluid, hemorrhage, calcification, or retinal traction. Fluorescein angiography disclosed mild hyperfluorescence in the venous phase and moderate late staining of the lesions. Autofluorescence showed mild hyperautofluorescence of the lesions. Ultrasonography revealed no calcification. Optical coherence tomography showed an abruptly elevated retinal mass with optical shadowing posterior to the lesion. Six lesions were stable after a median follow-up of 6 years, and 1 lesion gradually disappeared. The pathogenesis and pathologic features of PSCRAP are unknown. Conclusion: Presumed solitary circumscribed retinal astrocytic proliferation appears to be a unique retinal lesion of adulthood that resembles astrocytic hamartoma or retinoblastoma but displays distinctive ophthalmoscopic features.

Reactive Retinal Astrocytic Tumor (Focal Nodular Gliosis): Report of the Clinical Spectrum of 3 Cases

“…Related rare entities such as presumed solitary circumscribed retinal astrocytic proliferation [18,19,20] have clinical appearance of a white circumscribed, retinal lesion without prominent retinal vascularity in absence of associated subretinal fluid or hemorrhage and may be included within the spectrum of reactive retinal astrocytic tumors as well as acquired astrocytomas (not associated with tuberous sclerosis) that are composed of astrocytes lacking mitotic figures [21,22]. …”

Solitary Circumscribed “Pearl White” Retinal Mass (So-Called Retinal Astrocytic Proliferation) Resides in Deep Retina or Beneath Retina: Findings on Multimodal Imaging in 4 Cases

“…[1][2][3][4] Other nonneoplastic retinal conditions that arise from glial cells include reactive gliosis after retinal damage, massive gliosis after extensive retinal disease, and solitary circumscribed retinal astrocytic proliferation. [1][2][3][4][5][6][7] In 2011, Shields et al 2 published the initial clinical description of solitary circumscribed retinal astrocytic proliferation, identifying that this yellow-white "pearly" retinal lesion appeared distinctly circumscribed, abruptly elevated on the inner retinal surface, obscuring retinal vessels, and remaining stable. There was no relationship with tuberous sclerosis complex.…”

“…2 They speculated that this lesion might represent focal astrocytic proliferation on the inner retina. 2 Notably on time-domain optical coherence tomography, the mass tented the retinal margins, but resolution was insufficient to determine level of the mass within the retina.…”

“…2 They speculated that this lesion might represent focal astrocytic proliferation on the inner retina. 2 Notably on time-domain optical coherence tomography, the mass tented the retinal margins, but resolution was insufficient to determine level of the mass within the retina. 2 They commented that "all lesions were located in the sensory retina, presumably the nerve fiber layer, and it was not possible to determine how far they extended into the deeper retinal layers."…”

“…2 Notably on time-domain optical coherence tomography, the mass tented the retinal margins, but resolution was insufficient to determine level of the mass within the retina. 2 They commented that "all lesions were located in the sensory retina, presumably the nerve fiber layer, and it was not possible to determine how far they extended into the deeper retinal layers." In this report, we have evaluated four additional cases with spectral domain optical coherence tomography (SD-OCT) and have recognized that solitary circumscribed retinal astrocytic proliferation appears to be predominantly localized in the deep retina or at the retinal pigment epithelial (RPE) level.…”