Abstract
SUMMARY Von Hippel-Landau disease is a hereditary syndrome autosomal dominant associated to a suppresor gene mutation in chromosome 3p25-26 with attendant genetic risk of hemangioblastoma formation in the brain, spinal cord and retina, pheocromocytoma, renal cells carcinoma, endolymphatic sac tumours, and renal, pancreatic, hepatic, broad ligament or epididymal cyst. We present a clinical case of espinal hemangioblastoma with diagnosis during the first pregnancy. The tumor was surgical treated after delivery and she developed another pregnancy afterward. We discuss general aspects of hemangioblastoma in Central Nervous System and others localizations and its pregnancy relation. There is a new clasification of syndrome and we discuss the actual management.
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