title: Long-term prognosis of haemangioblastoma of the CNS: impact of von Hippel-Lindau disease.
abstract: The aim was to assess the frequency of von Hippel-Lindau disease (VHL) and the long-term prognosis of VHL and non-VHL patients among 110 consecutive patients with haemangioblastoma (HB) of the CNS treated between 1953 and 1993 at one neurosurgical unit. To reveal VHL manifestations we performed a detailed clinical and radiological examination (neuraxis and abdomen) (61/110), VHL-gene mutation analysis (40/110), and collection of all available clinical, imaging, operative and autopsy data from the hospitals involved. All patients were followed-up with a median of 14 years (excluding 14 operative deaths), and no patient was lost to follow-up. Altogether 49 patients died during the follow-up. In the 14 VHL patients (13%), HB(s) of the CNS were detected at a median age of 33 years, retinal HB(s) at 39 years, and renal cell carcinoma (RCC) at 43 years. The frequency of VHL in patients operated on for HB(s) was 29% before the age of 25 years, 19% between 25 and 45 years, and only 2% after 45 years. HB patients not meeting the VHL criteria had internal organ cysts in 14%. One non-VHL patient (4%) had two adjacent HBs in the same cyst wall. The growth rates of non-VHL and VHL-related HBs were similar as indicated by the median time to recurrence and the proliferation indices (MIB-1). Recurrence of the HB in patients whose primary operation was considered radical developed in four of the 10 VHL patients at a median of 19 years, and in nine of the 74 non-VHL patients at a median of 11 years. The median length of life of all VHL and non-VHL patients was 46 and 63 years, respectively. In VHL, RCC and HBs were equal causes of death.
authors.affiliation: Department of Neurosurgery, Helsinki University Hospital, Helsinki, Finland.
authors.forename: M
authors.initials: M
authors.lastname: Niemelä
authors.forename: S
authors.initials: S
authors.lastname: Lemeta
authors.forename: P
authors.initials: P
authors.lastname: Summanen
authors.forename: T
authors.initials: T
authors.lastname: Böhling
authors.forename: M
authors.initials: M
authors.lastname: Sainio
authors.forename: J
authors.initials: J
authors.lastname: Kere
authors.forename: K
authors.initials: K
authors.lastname: Poussa
authors.forename: R
authors.initials: R
authors.lastname: Sankila
authors.forename: H
authors.initials: H
authors.lastname: Haapasalo
authors.forename: H
authors.initials: H
authors.lastname: Kääriäinen
authors.forename: E
authors.initials: E
authors.lastname: Pukkala
authors.forename: J
authors.initials: J
authors.lastname: Jääskeläinen
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D016543:Central Nervous System Neoplasms / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D003952:Diagnostic Imaging; D005260:Female; D005500:Follow-Up Studies; D018325:Hemangioblastoma / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000235:genetics / Q000401:mortality / Q000601:surgery; D012086:Reoperation; D015996:Survival Rate; D006623:von Hippel-Lindau Disease / Q000235:genetics / Q000401:mortality / Q000601:surgery*