Long-term natural history of neurofibromatosis Type 2-associated intracranial tumors.
Long-term natural history of neurofibromatosis Type 2-associated intracranial tumors.
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DOI：
10.3171/2012.3.jns111649
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发表时间：
2012-07
影响因子：
4.1
通讯作者：
Asthagiri AR
中科院分区：
文献类型：
--
作者：
Dirks MS;Butman JA;Kim HJ;Wu T;Morgan K;Tran AP;Lonser RR;Asthagiri AR
2型神经纤维瘤病（NF 2）是一种遗传性肿瘤易感综合征，可导致多发性颅内肿瘤的发生，包括脑膜瘤和神经鞘瘤。由于这些肿瘤的自然史尚未确定，因此尚未建立其最佳管理。为了明确NF 2相关颅内肿瘤的自然史并优化治疗策略，作者评价了NF 2患者的长期临床和影像学数据。对连续4年临床和MRI随访的连续性NF 2患者进行了分析。17名患者（9名男性和8名女性）被纳入该分析（平均随访时间为9.5 ± 4.8年，范围为4.0-20.7年）。初次评价时的平均年龄为33.2 ± 15.5岁（范围12.3-57.6岁）。患者有182个颅内肿瘤，其中164个可用于生长率分析（18个前庭神经鞘瘤[VS]，11个非前庭脑神经[CN]神经鞘瘤和135个脑膜瘤），其中152个可用于生长模式分析（15个VS，9个非前庭CN神经鞘瘤和128个脑膜瘤）。在影像学随访过程中，患者发生了新的肿瘤：66例脑膜瘤，2例VS和2例非前庭CN神经鞘瘤。总体而言，45例肿瘤（29.6%）表现出线性生长，17例肿瘤（11.2%）表现出指数生长，90例肿瘤（59.2%）表现出跳跃生长模式，其特征为生长期和静止期交替（平均静止期2.3 ± 2.1年，范围0.4-11.7年）。此外，跳跃型是每种肿瘤类型最常见的生长模式：脑膜瘤60.9%，VS 46.7%，非前庭神经鞘瘤55.6%。较年轻的NF 2相关症状的发病年龄（p = 0.01）和女性（p = 0.05）与脑膜瘤的生长速度增加相关。随着随访时间的延长，脑膜瘤跳跃性生长的识别率增加（p = 0.01）。2型神经纤维瘤病相关颅内肿瘤最常表现为跳跃性生长模式。由于NF 2患者在其一生中可能会发生新的肿瘤，并且由于放射学进展和症状形成是不可预测的，因此切除术可能最好保留给产生肿瘤的患者。此外，非手术治疗干预的疗效必须建立在长期随访（数年）的基础上。
Neurofibromatosis Type 2 (NF2) is a heritable tumor predisposition syndrome that leads to the development of multiple intracranial tumors, including meningiomas and schwannomas. Because the natural history of these tumors has not been determined, their optimal management has not been established. To define the natural history of NF2-associated intracranial tumors and to optimize management strategies, the authors evaluated long-term clinical and radiographic data in patients with NF2. Consecutive NF2 patients with a minimum of 4 years of serial clinical and MRI follow-up were analyzed. Seventeen patients, 9 males and 8 females, were included in this analysis (mean follow-up 9.5 ± 4.8 years, range 4.0–20.7 years). The mean age at initial evaluation was 33.2 ± 15.5 years (range 12.3–57.6 years). Patients harbored 182 intracranial neoplasms, 164 of which were assessable for growth rate analysis (18 vestibular schwannomas [VSs], 11 nonvestibular cranial nerve [CN] schwannomas, and 135 meningiomas) and 152 of which were assessable for growth pattern analysis (15 VSs, 9 nonvestibular CN schwannomas, and 128 meningiomas). New tumors developed in patients over the course of the imaging follow-up: 66 meningiomas, 2 VSs, and 2 nonvestibular CN schwannomas. Overall, 45 tumors (29.6%) exhibited linear growth, 17 tumors (11.2%) exhibited exponential growth, and 90 tumors (59.2%) displayed a saltatory growth pattern characterized by alternating periods of growth and quiescence (mean quiescent period 2.3 ± 2.1 years, range 0.4–11.7 years). Further, the saltatory pattern was the most frequently identified growth pattern for each tumor type: meningiomas 60.9%, VSs 46.7%, and nonvestibular schwannoma 55.6%. A younger age at the onset of NF2-related symptoms (p = 0.01) and female sex (p = 0.05) were associated with an increased growth rate in meningiomas. The identification of saltatory growth in meningiomas increased with the duration of follow-up (p = 0.01). Neurofibromatosis Type 2–associated intracranial tumors most frequently demonstrated a saltatory growth pattern. Because new tumors can develop in NF2 patients over their lifetime and because radiographic progression and symptom formation are unpredictable, resection may be best reserved for symptom-producing tumors. Moreover, establishing the efficacy of nonsurgical therapeutic interventions must be based on long-term follow-up (several years).