Familial Neoplastic Syndromes.

Familial neoplastic syndromes commonly impact the central and peripheral nervous systems. The most common neoplastic syndromes clinically relevant to neurology and neurologic surgery, include neurofibromatosis type 1, neurofibromatosis type 2, and Von Hippel-Lindau disease. We define the epidemiology, genetics, clinical presentation, and manifestations, as well as screening recommendations and management paradigms for these syndromes and other familial neoplastic syndromes that affect the nervous system. To ensure the optimal care of patients with neoplastic syndromes requires a multi-disciplinary approach and a comprehensive knowledge of the genetics, pathophysiology, manifestations, and management.