Reactive Retinal Astrocytic Tumor (Focal Nodular Gliosis): A Case Report

Abstract

Purpose: To report the clinical and histopathological findings of a reactive retinal astrocytic tumor (RRAT) that progressed to massive retinal gliosis. Observations: The patient presented with an elevated, white-yellow retinal mass and extensive retinal exudation in the left eye. Progressive enlargement of the mass and proliferative vitreoretinopathy eventually led to phthisis bulbi and enucleation. Histologically, the mass showed a predominant astrocytic component with intense glial fibrillary acidic protein staining, hyperplasia, fibrous metaplasia, and osseous metaplasia of the retinal pigment epithelium. The Ki-67 proliferative index was <5%, and few scattered vascular channels were observed. Conclusions and Importance: These findings show that this tumor is the result of a reactive glial process rather than of neoplastic vascular proliferation. Massive retinal gliosis probably represents the advanced stage of RRAT.

Orbital tumor associated with a microphthalmic eye and colobomatous cleft: Pilocytic astrocytoma (glioma) or massive retinal gliosis

“…A boy with congenital unilateral microphthalmos presented with an orbital mass with histologic features of pilocytic astrocytoma and/or retinal gliosis. Using light microscopy, low-grade intraocular astrocytoma is indistinguishable from massive retinal gliosis 1, 2, 3, 4…”

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

“…The findings in our patients are consistent with the reactive nature of this tumor, which may occur in a variety of settings. These tumors are slow growing, variably vascularized, and may eventually be classified as massive gliosis of the retina [17]. …”

Retinal Reactive Astrocytic Tumor (Focal Nodular Gliosis): The Entity Also Known as Vasoproliferative Tumor

“…These iterative name changes often coincide with histopathologic and molecular diagnostic interpretations of clinically described entities. As has occurred many times in the past, such as for the retinal tumors retinoblastoma and hemangioblastoma, a name change is currently underway for the retinal tumor reactive retinal astrocytic tumor (focal nodular gliosis), formerly known as vasoproliferative tumor [1,2,3]…”

“…They suggested that these lesions are all interrelated and exist on a continuous spectrum [2]. Hudson and co-workers [3] published the “missing link” case, in which a patient first clinically diagnosed with a “retinal angioma” (focal nodular gliosis) was followed over a period of years during which the tumor slowly progressed; the eye was subsequently enucleated and it was shown to contain massive gliosis. Taken together, it is now clear that retinal reactive astrocytic tumor (focal nodular gliosis) is the preferred name for this entity.…”