Hemangioblastomas are intracranial and intraspinal tumors arising sporadica
lly or in patients with von Hippel-Lindau disease. To date, hemangioblastom
as have not been described in the skin. Proliferating clear cells with a va
riable vascular component are found histologically. These clear cells stain
for neuron-specific enolase but not cytokeratin or epithelial membrane ant
igen, allowing them to be differentiated from metastatic renal cell carcino
ma.