Optic nerve astrocytoma associated with aegiogenesis and neovascular glaucoma

A 10-year-old boy with a history of neurofitoromatosis type 1 presented with visual loss and proptosis in his left eye. Neuroimaging revealed a fusiform, enhancing mass of the left optic nerve extending through the optic canal and involving the intracranial portion of the nerve. Five months later, he experienced acute onset of severe pain and a dilated left pupil. Examination disclosed an increase in proptosis from 4 to 9 mm, neovascular glaucoma, and a central retinal vein occlusion. Repeat imaging showed a marked increase in the size of the mass. The left eye and the intraorbital portion of the optic nerve tumor were resected en bloc. Histologic examination showed a pilocytic astrocytoma of the optic nerve with a prominent angiogenic response, iris neovascularization, and angle closure. This case illustrates the rare occurrence of prominent angiogenic response and neovascular glaucoma complicating a pilocytic astrocytoma of the optic nerve.

Duke Scholars

Author Thomas John Cummings Pathology