Natural History of Vestibular Schwannomas in Neurofibromatosis Type 2 (FN2)

摘要

Neurofibromatosis 2 (NF2) is an autosomal disorder characterized by the development of multiple nervous system tumors such as vestibular schwannomas. The purpose of the study is to define the growth rate and clinical course of vestibular schwannomas in NF2- affected individuals. We will develop an international consortium of clinical centers with expertise in NF2, standardize the radiographic analysis of the vestibular schwannomas, assess the patients' audiological functioning, and analyze molecular, pathological, and clinical features of the disease over the course of 3 years. We have enrolled nearly 50% of the target goal of 100 study participants. The difficulty enrolling patients has stemmed from a protracted informed consent approval process, one co-Pi leaving a core site, and a lengthy process to ensure that MRI scanning facilities are compatible with WorldCare's systems. We have addressed the enrollment problem by lengthening the enrollment window to January 31, 2000 and expanded the inclusion criteria to include NF2-affected family members. Baseline data have been collected on 65% and 52% of the enrolled patients for audiological and MRI data respectively. We have made good progress toward completion of the first year's goals and anticipate few problems with the collection of 1 year follow-up data.