Clinicopathologic correlation of choroidal folds: secondary to massive cranioorbital hemangiopericytoma

A 55-year-old woman developed marked choroidal folds in the right eye secondary to a massive tumor in the right orbit. The patient underwent a modified exenteration with debulking of the tumor, thus providing the opportunity to do a clinicopathologic correlation on the choroidal folds. Histopathologically, the orbital tumor proved to be an unusually aggressive malignant hemangiopericytoma. Microscopic examination of the globe showed that the apices of some of the folds were directed toward the retina, whereas the apices of other folds were directed toward the choroid. In contrast to popular concepts, the retinal pigment epithelium was intact at the crests of the folds toward the retinal side, while the retinal pigment epithelium was atrophic in the valleys toward the choroid.