*Department of Neurology and Cancer Center, †Biostatistics Center, and ‡Neurosurgical Service, Massachusetts General Hospital, Boston, Massachusetts; and §Neurotology, House Clinic, Los Angeles, California, U.S.A.
Otol Neurotol. 2014 Jan;35(1):e50-6. doi: 10.1097/MAO.0000000000000239.
To determine the rate of growth in vestibular schwannomas and the rate of hearing decline in neurofibromatosis type 2 (NF2) patients not undergoing active treatment
Prospective study.
Data were collected at 10 NF2 centers, including hospital-based, academic, and tertiary care centers.
120 NF2 patients with 200 vestibular schwannomas.
Hearing decline, defined as a decrease in word recognition score outside the 95% critical difference compared with baseline, and radiographic progression, defined as a 20% or greater increase in tumor volume compared with baseline.
During a total of 313.4 patient-years of follow-up, the rate of hearing decline was 5% at 1 year, 13% at 2 years, and 16% at 3 years; the rate of tumor progression was 31% at 1 year, 64% at 2 years, and 79% at 3 years. For this cohort, the median time to tumor progression (14 mo) was significantly shorter than the median time to hearing decline (62.0 mo).
These data provide potentially useful information for the design of clinical trials for NF2 vestibular schwannoma.
确定未经积极治疗的神经纤维瘤病 2 型(NF2)患者前庭神经鞘瘤的生长率和听力下降率。
前瞻性研究。
数据收集于 10 个 NF2 中心,包括医院、学术和三级保健中心。
120 名 NF2 患者,200 个前庭神经鞘瘤。
听力下降,定义为与基线相比,言语识别得分下降超过 95%的临界差异;影像学进展,定义为与基线相比肿瘤体积增加 20%或以上。
在总共 313.4 名患者年的随访中,1 年、2 年和 3 年的听力下降率分别为 5%、13%和 16%;肿瘤进展率分别为 1 年时为 31%、2 年时为 64%和 3 年时为 79%。对于该队列,肿瘤进展的中位时间(14 个月)明显短于听力下降的中位时间(62.0 个月)。
这些数据为 NF2 前庭神经鞘瘤的临床试验设计提供了有潜在价值的信息。