AUTHOR=Schunemann Victoria , Huntoon Kristin , Lonser Russell R. TITLE=Personalized Medicine for Nervous System Manifestations of von Hippel–Lindau Disease JOURNAL=Frontiers in Surgery VOLUME=3 YEAR=2016 URL=https://www.frontiersin.org/journals/surgery/articles/10.3389/fsurg.2016.00039 DOI=10.3389/fsurg.2016.00039 ISSN=2296-875X ABSTRACT=
von Hippel–Lindau disease (VHL) is a familial neoplasia syndrome associated with multisystem tumor development. Depending on tumor type and location, current treatments for VHL-associated tumors can include a combination of chemotherapy, radiation therapy, and/or surgery. Central nervous system (CNS) manifestations of VHL include craniospinal hemangioblastomas and endolymphatic sac tumors (ELSTs). While the first-line treatment for both types of VHL-associated CNS tumors is surgery, the indications for treatment are patient specific and different for each tumor type. Although early sign/symptom formation is the primary indication for resection of craniospinal hemangioblastomas, radiographic discovery (asymptomatic and symptomatic) of ELSTs can be an indication for resection of ELSTs in VHL patients. Recently, research has revealed that specific