Long-term prognosis of hemangioblastomas of the central nervous system: clinical and immunohistochemical study in relation to recurrence.

The long-term prognosis and immunohistochemical findings for the expression of VEGF (vascular endothelial growth factor), p53 protein, and proliferative potential with MIB-1 were evaluated in six patients with VHL (von Hippel-Lindau) disease and seven patients with sporadic hemangioblastomas in relation to recurrent or new central nervous system (CNS) hemangioblastomas following treatment. Sporadic CNS hemangioblastomas were treated by total removal, and they demonstrated a good long-term prognosis without neurological deficits and no recurrence. However, even if total removal of the CNS hemangioblastomas in patients with VHL disease was performed initially, new lesions, consisting of small multiple hemangioblastomas, recurred in areas remote from the primary region in three of four patients during long-term follow-up. Such newly developed hemangioblastomas in patients with VHL disease could reflect the biological multipotentiality of CNS hemangioblastomas that were not detected during the initial operation. All of the hemangioblastomas displayed extensive overexpression of VEGF protein. The immunohistochemical findings for VEGF protein, p53 protein, and MIB-1 did not differ significantly between the sporadic and VHL disease-associated hemangioblastomas. In conclusion, since patients with VHL disease are at risk of developing new lesions, the management of hemangioblastomas in patients with VHL disease represents a more prolonged and difficult task.