Solitary peripapillary hemangioblastoma - A histopathological case report

Purpose: Hemangioblastomas are highly vascularized tumors of not well-defin ed histological origin. They are the most frequent manifestation of the von -Hippel-Lindau (VHL) disease, but also occur as sporadic non-hereditary tum ors 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 i n a 58-year-old patient. Results: Histological examination demonstrated a highly vascularized tumor with intercapillary stromal tells. The tumor did not invade the adjacent ti ssues. Surrounding retinal areas showed cystic and gliotic changes. Immunoh istochemistry was unable to prove neuroglial origin of the stromal cells. Conclusion: Histological findings show similarities to the findings in cere bral hemangioblastomas. The tumor location in this case together with the k nown response of glial cells to VHL, might indicate their involvement in th e pathogenesis of hemangioblastoma.