Liu James K, Forman Scott, Hershewe Gerard L, Moorthy Chitti R, Benzil Deborah L
Department of Neurosurgery, New York Medical College, Valhalla, New York 10595, USA.
Neurosurgery. 2002 May;50(5):950-5; discussion 955-7. doi: 10.1097/00006123-200205000-00006.
The management of primary optic nerve sheath meningioma (ONSM) is controversial. Surgery often results in postoperative blindness in the affected eye and thus has been abandoned as a treatment option for most patients. When these tumors are left untreated, however, progressive visual impairment ensues, which also leads to blindness. Recently, radiation therapy has gained wider acceptance in the treatment of these lesions. Experience with stereotactic radiotherapy (SRT) in the treatment of ONSMs is limited because of the rare incidence of this tumor. We present a series of patients with ONSM who were treated with SRT.
Five patients (three women, two men), ranging in age from 40 to 73 years, presented with progressive visual loss with decreased visual field, visual acuity, and color vision affecting six eyes (one patient had tumor involving both optic nerves). One patient also presented with proptosis and diplopia. Five eyes had functional residual vision (range, 20/20 to 20/40), and one eye was completely blind. All five patients were diagnosed clinically and radiographically to have an ONSM. Three were intraorbital, one was intracanalicular as well as intraorbital, and one was a left ONSM extending through the optic foramen into the intracranial space and involving the right optic nerve. The five functional eyes were treated with SRT by use of 1.8-Gy fractions to a cumulative dose of 45 to 54 Gy.
Follow-up ranged from 1 to 7 years, and serial magnetic resonance imaging revealed no changes in the size of the tumor in all five patients. Four patients experienced dramatic improvement in visual acuity, visual field, and color vision within 3 months after SRT. One patient remained stable without evidence of visual deterioration or disease progression. None had radiation-induced optic neuropathy.
SRT may be a viable option for treatment of primary ONSM in patients with documented progressive visual deterioration, and it may be effective in improving or stabilizing remaining functional vision.
原发性视神经鞘膜瘤（ONSM）的治疗存在争议。手术常常导致患眼术后失明，因此大多数患者已放弃将手术作为一种治疗选择。然而，当这些肿瘤不进行治疗时，会出现进行性视力损害，最终也会导致失明。最近，放射治疗在这些病变的治疗中得到了更广泛的认可。由于这种肿瘤发病率低，立体定向放射治疗（SRT）治疗ONSM的经验有限。我们报告一组接受SRT治疗的ONSM患者。
5例患者（3例女性，2例男性），年龄40至73岁，均出现进行性视力丧失，伴有视野、视力和色觉下降，累及6只眼（1例患者肿瘤累及双侧视神经）。1例患者还出现眼球突出和复视。5只眼有功能性残余视力（范围为20/20至20/40），1只眼完全失明。所有5例患者经临床和影像学诊断均为ONSM。3例为眶内型，1例为管内及眶内型，1例为左侧ONSM，经视神经孔延伸至颅内空间并累及右侧视神经。对5只具有功能性视力的眼睛采用SRT治疗，每次剂量1.8 Gy，累积剂量45至54 Gy。
随访时间为1至7年，连续磁共振成像显示所有5例患者肿瘤大小均无变化。4例患者在SRT治疗后3个月内视力、视野和色觉显著改善。1例患者病情稳定，无视力恶化或疾病进展迹象。均未发生放射性视神经病变。
对于有记录的进行性视力恶化的原发性ONSM患者，SRT可能是一种可行的治疗选择，并且可能有效改善或稳定残余的功能性视力。
