Choroidal ganglioneuroma in neurofibromatosis.

Daniel Albert // Publications // Jan 01 1983

PubMed ID: 6403410

Author(s): Woog JJ, Albert DM, Craft J, Silberman N, Horns D. Choroidal ganglioneuroma in neurofibromatosis. Graefes Arch Clin Exp Ophthalmol. 1983;220(1):25-31. PMID 6403410

Journal: Graefe’s Archive For Clinical And Experimental Ophthalmology = Albrecht Von Graefes Archiv Fur Klinische Und Experimentelle Ophthalmologie, Volume 220, Issue 1, 1983

A twenty-one year old female with previously unsuspected neurofibromatosis presented for evaluation of a blind painful eye. Histopathologic examination of the enucleated specimen revealed choroidal thickening with ovoid bodies and proliferation of connective tissue with pigment-containing cells and ganglion-like cells. Electron microscopic study of the latter cell population revealed typical morphologic features of ganglion cells, including numerous electron-dense intracellular granules and an abundance of mitochondria and rough endoplasmic reticulum. These pathologic findings were interpreted as consistent with the diagnosis of choroidal ganglioneuroma occurring in the context of ocular neurofibromatosis. The literature concerning this unusual tumor is reviewed and the possible relationship of this lesion to neurofibromatosis and other disorders of neural cresent proliferation are briefly discussed.