Von Hippel-Lindau syndrome is characterized by the development of hemangioblastomas in the brain, spinal cord and retina. Likewise, to the appearance of renal cysts and clear cell renal carcinoma, to the development of pheochromocytoma and paraganglioma, pancreatic cysts and neuroendocrine tumors, endolymphatic sac tumors and epididymal cystadenomas.
Cerebellar hemangioblastomas may be associated with headache, vomiting, gait disturbances, or ataxia. Spinal hemangioblastomas usually present with pain. Sensory and motor loss can develop with compression of the spinal cord.