Neuropathology of von Hippel-Lindau Disease - Abstract
VHL patients develop tumors in brain, spinal cord, nerve roots, kidney, adrenal gland and extra-adrenal ganglia, pancreas, epididymis and broad ligament. VHL disease produces highly characteristic neuropathologic changes which are presented in this review. Highly vascularized tumors, hemangioblastomas, involve cerebellum and spinal cord of the majority of patients. A smaller number of patients may develop endolymphatic sac tumors that originate in the petrous bone, but may extend into the brain. Lastly, neuropathology may be produced by metastasis of renal clear cell carcinoma or other VHL disease-associated neoplasms.