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Medline ® Abstract for Reference 95

of 'Clinical features, diagnosis, and management of von Hippel-Lindau disease'

95
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Effect of pregnancy on hemangioblastoma development and progression in von Hippel-Lindau disease.
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Ye DY, Bakhtian KD, Asthagiri AR, Lonser RR
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J Neurosurg. 2012 Nov;117(5):818-24. Epub 2012 Aug 31.
 
Object 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.
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Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.
PMID