title: Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study.
abstract: OBJECTIVE
  The purpose of this study was to evaluate the role of stereotactic radiosurgery (SRS) in the management of intracranial hemangioblastomas.
  
  
  METHODS
  Six participating centers of the North American Gamma Knife Consortium and 13 Japanese Gamma Knife centers identified 186 patients with 517 hemangioblastomas who underwent SRS. Eighty patients had 335 hemangioblastomas associated with von Hippel-Lindau disease (VHL) and 106 patients had 182 sporadic hemangioblastomas. The median target volume was 0.2 cm(3) (median diameter 7 mm) in patients with VHL and 0.7 cm(3) (median diameter 11 mm) in those with sporadic hemangioblastoma. The median margin dose was 18 Gy in VHL patients and 15 Gy in those with sporadic hemangioblastomas.
  
  
  RESULTS
  At a median of 5 years (range 0.5-18 years) after treatment, 20 patients had died of intracranial disease progression and 9 patients had died of other causes. The overall survival after SRS was 94% at 3 years, 90% at 5 years, and 74% at 10 years. Factors associated with longer survival included younger age, absence of neurological symptoms, fewer tumors, and higher Karnofsky Performance Status. Thirty-three (41%) of the 80 patients with VHL developed new tumors and 17 (16%) of the106 patients with sporadic hemangioblastoma had recurrences of residual tumor from the original tumor. The 5-year rate of developing a new tumor was 43% for VHL patients, and the 5-year rate of developing a recurrence of residual tumor from the original tumor was 24% for sporadic hemangioblastoma patients. Factors associated with a reduced risk of developing a new tumor or recurrences of residual tumor from the original tumor included younger age, fewer tumors, and sporadic rather than VHL-associated hemangioblastomas. The local tumor control rate for treated tumors was 92% at 3 years, 89% at 5 years, and 79% at 10 years. Factors associated with an improved local tumor control rate included VHL-associated hemangioblastoma, solid tumor, smaller tumor volume, and higher margin dose. Thirteen patients (7%) developed adverse radiation effects (ARE) after SRS, and one of these patients died due to ARE.
  
  
  CONCLUSIONS
  When either sporadic or VHL-associated tumors were observed to grow on serial imaging studies, SRS provided tumor control in 79%-92% of tumors.
authors: Departments of 1Neurological Surgery, University of Pittsburgh, Pennsylvania;
authors: Hideyuki
authors: H
authors: Kano
authors: 2Yokohama Rosai Hospital, Yokohama;
authors: Takashi
authors: T
authors: Shuto
authors: 3Osaka City General Hospital, Osaka;
authors: Yoshiyasu
authors: Y
authors: Iwai
authors: 4University of Virginia, Charlottesville, Virginia;
authors: Jason
authors: J
authors: Sheehan
authors: 5Katsuta Hospital Mito GammaHouse, Hitachinaka;
authors: Masaaki
authors: M
authors: Yamamoto
authors: 6Barrow Neurological Institute, Phoenix, Arizona;
authors: Heyoung L
authors: HL
authors: McBride
authors: 7Kitanihon Hospital, Gosen;
authors: Mitsuya
authors: M
authors: Sato
authors: 8Tsukiji Neurological Clinic, Tokyo;
authors: Toru
authors: T
authors: Serizawa
authors: 9Saitama Gamma Knife Center, Saitama;
authors: Shoji
authors: S
authors: Yomo
authors: 10Mominoki Hospital, Kochi;
authors: Akihito
authors: A
authors: Moriki
authors: 11Asanogawa General Hospital, Kanazawa;
authors: Yukihiko
authors: Y
authors: Kohda
authors: 12University of Kentucky, Lexington, Kentucky;
authors: Byron
authors: B
authors: Young
authors: 13Steel Memorial Yawata Hospital, Kitakyushyu;
authors: Satoshi
authors: S
authors: Suzuki
authors: 14Nagatomi Neurosurgical Hospital, Oita;
authors: Hiroyuki
authors: H
authors: Kenai
authors: 15Hoag Hospital, Newport Beach, California;
authors: Christopher
authors: C
authors: Duma
authors: 16Southern Tohoku Hospital, Koriyama;
authors: Yasuhiro
authors: Y
authors: Kikuchi
authors: 17Université de Sherbrooke, Centre de Recherche Clinique Étienne-LeBel, Sherbrooke, Quebec, Canada; and.
authors: David
authors: D
authors: Mathieu
authors: 18NTT Kanto Hospital, Tokyo;
authors: Atsuya
authors: A
authors: Akabane
authors: 19Chiba Cardiovascular Center, Ichihara, Japan;
authors: Osamu
authors: O
authors: Nagano
authors: 20New York University Langone Medical Center, New York, New York.
authors: Douglas
authors: D
authors: Kondziolka
authors: Departments of 1Neurological Surgery, University of Pittsburgh, Pennsylvania;
authors: L Dade
authors: LD
authors: Lunsford
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001932:Brain Neoplasms / Q000473:pathology / Q000601:surgery*; D018450:Disease Progression; D005260:Female; D018325:Hemangioblastoma / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery*; D017063:Outcome Assessment, Health Care; D016634:Radiosurgery / Q000295:instrumentation*; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000473:pathology / Q000601:surgery*
keywords: ARE = adverse radiation effects; Gamma Knife; KPS = Karnofsky Performance Status; NAGKC = North American Gamma Knife Consortium; SRS = stereotactic radiosurgery; UPMC = University of Pittsburgh Medical Center; UVA = University of Virginia; VHL = von Hippel–Lindau disease; hemangioblastoma; stereotactic radiosurgery; von Hippel–Lindau disease