Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease.

[von hippel-lindau disease]

Two patients who had received a total resection of cerebellar hemangioblastoma developed cerebrospinal fluid dissemination during a long -term follow-up period . We present this rare disease with discussion based on the literature .The patients were two women aged 45 and 57 years . In the cerebellar hemisphere , one patient had cystic hemangioblastoma of mural nodule type and the other had solid type . Both the patients successfully underwent total resection by craniotomy . They presented no mutations in the von Hippel-Lindau disease (VHL ) gene or lesions in the other organs . One patient developed local recurrence 38 months after the initial surgery , and received stereotactic radiosurgery . Three spinal cord tumors developed 91 months later , and the tumors were disseminated to the entire cerebrospinal cavity 107 months later . The other patient developed hydrocephalus 53 months after the initial surgery with tumor tissues disseminated in the intracranial subarachnoid space . The conditions of the two patients gradually aggravated despite treatment with ventriculo-peritoneal shunt and irradiation to the whole brain and whole spinal cord .Cerebrospinal fluid dissemination of cerebellar hemangioblastoma was found dominantly in non-VHL patients . The diagnosis was made 10 years after the initial surgery . Irradiation therapy was performed , but the patients died about 2 years after the diagnosis was given . Molecular targeted therapies including vascular proliferation suppression have been attempted lately , but no effective therapy has been established . Early diagnosis of dissemination as well as combination of aggressive excision and stereotactic radiosurgery are considered to be appropriate for current interventions .