Histogenesis of haemangioblastomas: an immunocytochemical and ultrastructural study in a case of von Hippel-Lindau syndrome.

The cerebellar, retinal, and one of the spinal haemangioblastomas in a case of von Hippel-Lindau syndrome were studied by immunocytochemistry and electron microscopy. The tumours were positive for neurone specific enolase and variably positive for somatostatin, pancreatic polypeptide, and bombesin. Electron microscopy of the cerebellar tumour showed secretory granules with an average diameter of 170 nm. This report is believed to be the first description of neurone specific enolase positivity and polypeptide hormones within the intervascular cells of haemangioblastomas. In the light of these findings it is suggested that haemangioblastomas are tumours of neuroectodermal origin, derived either from neural or neuroendocrine cells.