title: Natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
abstract: BACKGROUND
  Supratentorial hemangioblastomas are rare lesions, occurring either sporadically or in von Hippel-Lindau disease.
  
  
  OBJECTIVE
  Following recent advances in our understanding of the natural history of von Hippel-Lindau-associated cerebellar and spinal hemangioblastomas, we conducted a study of the natural history of supratentorial hemangioblastomas in von Hippel-Lindau disease.
  
  
  METHODS
  We reviewed a series of 18 supratentorial hemangioblastomas in 13 patients with von Hippel-Lindau disease. Clinical, genetic, and serial imaging data and operative records were analyzed.
  
  
  RESULTS
  Hemangioblastomas were most commonly seen in the temporal lobe. Only 6 tumors had a cyst at diagnosis or during follow-up, and only 6 patients had associated symptoms at presentation or during follow-up. The most frequent clinical presentations were intracranial hypertension and visual loss. Of 14 tumors with documented serial imaging, 13 demonstrated tumor growth. Rates and patterns of tumor growth were unique to each patient. The mechanism of cyst formation described in other locations was also demonstrated in the supratentorial region. Patterns of peritumoral edema and rate of cyst formation seemed to be influenced by the presence of anatomic barriers. Germline VHL mutation was identified in all patients, but no specific genotype-phenotype correlation was found, although a familial predisposition is suggested.
  
  
  CONCLUSIONS
  This series illustrates the wide variation in tumor locations, patterns of growth, and edema progression seen in supratentorial hemangioblastomas and adds to our knowledge of the natural history of hemangioblastomas.
authors.affiliation: Service de Neurochirurgie, Hôpital de Bicêtre, AP-HP, Le Kremlin-Bicêtre, France. matthieupeyre@hotmail.fr
authors.forename: Matthieu
authors.initials: M
authors.lastname: Peyre
authors.forename: Philippe
authors.initials: P
authors.lastname: David
authors.forename: Remy
authors.initials: R
authors.lastname: Van Effenterre
authors.forename: Patrick
authors.initials: P
authors.lastname: François
authors.forename: Myriam
authors.initials: M
authors.lastname: Thys
authors.forename: Evelyne
authors.initials: E
authors.lastname: Emery
authors.forename: Aimée
authors.initials: A
authors.lastname: Redondo
authors.forename: Philippe
authors.initials: P
authors.lastname: Decq
authors.forename: Nozar
authors.initials: N
authors.lastname: Aghakhani
authors.forename: Fabrice
authors.initials: F
authors.lastname: Parker
authors.forename: Marc
authors.initials: M
authors.lastname: Tadié
authors.forename: Catherine
authors.initials: C
authors.lastname: Lacroix
authors.forename: Ranjeev
authors.initials: R
authors.lastname: Bhangoo
authors.forename: Sophie
authors.initials: S
authors.lastname: Giraud
authors.forename: Stéphane
authors.initials: S
authors.lastname: Richard
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D005260:Female; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D018325:Hemangioblastoma / Q000235:genetics* / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000235:genetics* / Q000473:pathology* / Q000503:physiopathology