title: Effect of pregnancy on hemangioblastoma development and progression in von Hippel-Lindau disease.
abstract: OBJECTIVE
  Prior cases suggest that pregnancy increases the development and progression of CNS hemangioblastomas and/or peritumoral cysts. To determine the effect of pregnancy on CNS hemangioblastomas and peritumoral cysts, the authors prospectively evaluated serial clinical and imaging findings in patients with von Hippel-Lindau (VHL) disease who became pregnant and compared findings during pregnancy to findings in the same patients when they were not pregnant as well as to findings from a cohort of VHL patients who did not become pregnant.
  
  
  METHODS
  Female VHL disease patients enrolled in a prospective natural history study who were of reproductive age (16-35 years at study entrance) were included. Analysis of serial clinical and imaging findings was performed.
  
  
  RESULTS
  Thirty-six consecutive female VHL disease patients harboring 177 hemangioblastomas were included (mean follow-up [± SD] 7.5 ± 2.3 years). Nine patients (25%) became pregnant (pregnancy cohort). The mean rates of development of new hemangioblastomas and peritumoral cysts in these women during pregnancy (0.4 ± 0.4 tumors/year; 0.1 ± 0.2 cysts/year) did not differ significantly (p > 0.05) from the mean rates in the same group during nonpregnant periods (0.3 ± 0.4 tumors/year; 0.1 ± -0.1 cysts/year) or from the rate in the 27 patients who did not become pregnant (the no-pregnancy cohort: 0.3 ± 0.5 tumors/year; 0.1 ± 0.2 cysts/year). Hemangioblastoma growth rates were similar (p > 0.05) during pregnancy (mean 29.8% ± 42.7% increase in volume per year) compared with during nonpregnant periods (41.4% ± 51.4%) in the pregnancy cohort and the no-pregnancy cohort (34.3% ± 55.3%). Peritumoral cyst growth rates during pregnancy (571.0% ± 887.4%) were similar (p > 0.05) to those of the no-pregnancy cohort (483.9% ± 493.9%), but the rates were significantly higher for women in the pregnancy cohort during nonpregnant periods (2373.6% ± 3392.9%; p < 0.05 for comparison with no-pregnancy cohort). There was no significant difference (p > 0.05) in the need for resection or the mean age at resection between the pregnancy (28% of hemangioblastomas in cohort; mean patient age at resection 30.2 ± 2.6 years) and no-pregnancy cohorts (19%; 32.3 ± 5.6 years).
  
  
  CONCLUSIONS
  Pregnancy is not associated with increased hemangioblastoma or peritumoral cyst development or progression in patients with VHL disease.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
authors.forename: Donald Y
authors.initials: DY
authors.lastname: Ye
authors.forename: Kamran D
authors.initials: KD
authors.lastname: Bakhtian
authors.forename: Ashok R
authors.initials: AR
authors.lastname: Asthagiri
authors.forename: Russell R
authors.initials: RR
authors.lastname: Lonser
mesh_terms: D000293:Adolescent; D000328:Adult; D016543:Central Nervous System Neoplasms / Q000473:pathology* / Q000628:therapy; D015331:Cohort Studies; D003560:Cysts / Q000209:etiology / Q000473:pathology; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D018325:Hemangioblastoma / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008279:Magnetic Resonance Imaging; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000628:therapy*; D011446:Prospective Studies; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000473:pathology* / Q000628:therapy