Tumors of the cerebellum and IV. ventricles in adults

Hemangioblastoma

 * formerly called angioreticulum
 * in 80% it is a spontaneously occurring tumor
 * 20% are part of a genetically determined syndrome - von Hippel-Lindau syndrome (VHL) (CNS hemangiomas, retinal angiomatosis, cysts of parenchymatous organs)
 * 25% of them have kidney cancer, phaeochromocytoma and more often also pancreatic cancer!
 * is benign, mainly affects younger individuals
 * is most often in the cerebellum
 * is often cystic or may be solid, highly vascularized
 * it is the cystic form that is dangerous, because it can start to increase rapidly and is life-threatening with an acute occipital herniation!
 * clinical picture – cerebellar syndrome and intracranial hypertension syndrome
 * in 20%, polyglobulia from ectopic EPO production is a tumor
 * diagnosis - preferably on MRI with Gd contrast - cyst with hyperdense tumor nodule
 * therapy – cystic hemangioblastoma is always an indication for urgent surgery!, a cone can occur at any time and death can occur within minutes, it is necessary to look for VHL disease in the family, look for cysts on the organs with a sonogram, ...