von Hippel Angiomatosis

A 25-year-old Iranian man had undergone eye wall resection of a large von Hippel angioma to alleviate an exaggerated macular response, affording study by light and electron microscopy and immunohistochemistry before the obfuscatory effects of long-standing exudative retinal detachment, gliosis, or iatrogenic ablation supervened. We used this vantage point to assess the interrelation between the component endothelial cells, pericytes, and stromal foam cells. On the basis of staining with glial fibrillary acidic protein, factor VIII, the C3 fraction of complement, fibrinogen, and lysozyme, it is unlikely that stromal foam cells derive from glial precursors, but may represent degenerating cells, perhaps arising from a common vasoformative stem cell under hypoxic stress.