We examined the clinical characteristics and prognosis in six patients with familial von Hippel-Lindau (VHL) disease and seven with sporadic
hemangioblastomas. The expression of
vascular endothelial growth factor (
VEGF), p53
protein, and proliferative potential with Ki67
monoclonal antibody (MIB-1) was compared using immunohistochemical methods between sporadic and VHL disease-associated
hemangioblastomas. Patients with sporadic CNS
hemangioblastomas were treated by total removal of the
tumors, and they had a good long-term prognosis without neurological deficits on recurrence. However, patients with familial VHL disease often had
multiple hemangioblastomas in the CNS and visceral
tumors. Even if total removal of CNS
hemangioblastomas in patients with VHL disease was performed initially, small
multiple hemangioblastomas recurred during long-term follow-up in areas remote from the primary region resected by surgery. All of the
hemangioblastomas displayed extensive overexpression of
VEGF protein, with moderate to marked proliferation of blood vessels. The MIB-1 indices showed low values of 0.8% as the mean, with a range of 0.03%-2.1% for all the
hemangioblastomas. None of the
hemangioblastomas expressed p53
protein. The
hemangioblastomas in patients with VHL disease were multiple in the CNS and were combined with visceral
tumors. Patients with VHL disease had a poor long-term prognosis, in contrast to those with sporadic
hemangioblastomas. The immunohistochemical findings for
VEGF protein, p53
protein, and MIB-1 did not differ significantly between the sporadic and VHL disease-associated
hemangioblastomas.