Purpose: Hemangioblastomas are highly vascularized tumors of not well-defined histological origin. They are the most frequent manifestation of the von-Hippel-Lindau (VHL) disease, but also occur as sporadic non-hereditary tumors. Much has been learned in the recent past about the genetic background, however, the cellular origin of the tumor is still not resolved.
Methods: We report a rare case of sporadic peripapillary hemangioblastoma in a 58-year-old patient.
Results: Histological examination demonstrated a highly vascularized tumor with intercapillary stromal cells. The tumor did not invade the adjacent tissues. Surrounding retinal areas showed cystic and gliotic changes. Immunohistochemistry was unable to prove neuroglial origin of the stromal cells.
Conclusion: Histological findings show similarities to the findings in cerebral hemangioblastomas. The tumor location in this case together with the known response of glial cells to VHL, might indicate their involvement in the pathogenesis of hemangioblastoma.