Abstract
Neurofibromatosis 2 (NF2) and von Hippel-Lindau Disease (VHLD) are the two most frequently encountered familial neoplastic diseases in neurosurgery practice. Both cause multiple neoplasms in neural and extraneural systems and the patients have to undergo multiple surgeries during their lifetimes, each of which harbors the risk of incurring neurological deficits. Optimal management requires knowledge on the natural course of the disease, which is essential to determine the timing and method of intervention. In addition, an understanding of the genetic mechanism at the molecular level helps the physician make a more detailed diagnosis and create better follow-up schedules for each patient. However, genetic diagnosis can also have a profound impact on patients and their family members, and therefore, we should closely follow the guideline.
