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Medline ® Abstract for Reference 95

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

Effect of pregnancy on hemangioblastoma development and progression in von Hippel-Lindau disease.
Ye DY, Bakhtian KD, Asthagiri AR, Lonser RR
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.
Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.