title: Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease.
abstract: OBJECTIVE
  Despite the frequency of cerebellar hemangioblastomas in von Hippel-Lindau (VHL) disease, their optimum contemporary management has not been defined, and is made complex because of the multiple, progressive, and protean nature of the tumors found in patients with this disorder. To examine modern management and outcomes of cerebellar hemangioblastomas in VHL disease, the authors reviewed findings in patients with this disease who underwent resection of cerebellar hemangioblastomas.
  
  
  METHODS
  Consecutive patients with VHL disease who underwent surgery for cerebellar hemangioblastoma(s) at the National Institutes of Health were included. Eighty consecutive patients (44 female and 36 male patients) underwent 126 operations for removal of 164 cerebellar hemangioblastomas (age at surgery 37.8+/-10.3 years, follow-up duration 96.0+/-60.3 months). Serial clinical examinations, imaging studies, and operative records were analyzed.
  
  
  RESULTS
  Symptoms and signs included headache (94 operations; 75%), ataxia (55%), dysmetria (29%), and hydrocephalus (28%). Although the primary objective of surgery was resection of the hemangioblastoma considered responsible for symptoms (136 of the hemangioblastomas [83%]), 28 additional hemangioblastomas (17%) were removed during the same surgeries. Tumors associated with symptoms were larger (diameter 1.8+/-1.9 cm; volume 2.8+/-3.4 cm3; p<0.05) and more likely to be associated with peritumoral edema or peritumoral cysts (100% associated with edema and/or cyst; p<0.05) than asymptomatic tumors (diameter 1.1+/-0.9 cm; volume 0.7+/-0.4 cm3; 18%). More tumors were located in the posterior (74%) compared with the anterior (26%) half of the cerebellum (p<0.05). Three months after resection, symptom improvement/stabilization had occurred following 124 of the operations (98%). Preoperative hydrocephalus resolved after tumor removal in 33 cases (94%) and did not require cerebrospinal fluid diversion. Long-term imaging follow-up (61.5+/-15.0 months) revealed no recurrences.
  
  
  CONCLUSIONS
  Symptoms and signs caused by cerebellar hemangioblastomas in VHL disease are associated with edema and peritumoral cyst formation/propagation and are treated safely and effectively with resection. Cerebrospinal fluid diversion is rarely necessary after complete tumor removal in patients with preoperative hydrocephalus. Cerebellar hemangioblastomas are preferentially distributed in the posterior half of the cerebellum, as they are in the brainstem and spinal cord. Tumor recurrence is avoided by meticulous extracapsular resection.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
authors.forename: Jay
authors.initials: J
authors.lastname: Jagannathan
authors.forename: Russell R
authors.initials: RR
authors.lastname: Lonser
authors.forename: Rene
authors.initials: R
authors.lastname: Smith
authors.forename: Hetty L
authors.initials: HL
authors.lastname: DeVroom
authors.forename: Edward H
authors.initials: EH
authors.lastname: Oldfield
mesh_terms: D000328:Adult; D001259:Ataxia / Q000503:physiopathology; D001929:Brain Edema / Q000503:physiopathology; D002524:Cerebellar Ataxia / Q000503:physiopathology; D002528:Cerebellar Neoplasms / Q000503:physiopathology / Q000601:surgery*; D016371:Cranial Irradiation; D003560:Cysts / Q000503:physiopathology; D004210:Dissection / Q000379:methods; D004564:Electrocoagulation / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006261:Headache / Q000503:physiopathology; D018325:Hemangioblastoma / Q000503:physiopathology / Q000601:surgery*; D006801:Humans; D006849:Hydrocephalus / Q000503:physiopathology; D008297:Male; D042282:Microdissection / Q000379:methods; D011183:Postoperative Complications; D016634:Radiosurgery; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D016896:Treatment Outcome; D006623:von Hippel-Lindau Disease / Q000150:complications* / Q000503:physiopathology