视神经鞘脑膜瘤的临床诊疗及研究进展

Progress in diagnosis and treatment of optic nerve sheath meningiomas

视神经鞘脑膜瘤(optic nerve sheath meningioma,ONSM)是一种罕见且生长缓慢的良性肿瘤,起源于蛛网膜上皮细胞,较多见于中年女性。临床表现主要为无痛性进行性视力下降。诊断通常需要结合临床和影像学检查结果,CT/MRI的典型表现为视神经呈管状或梭形增粗,增强扫描后呈“双轨征”,可伴有钙化。治疗包括观察、放射治疗和手术治疗,其中放疗是首选方式。目前ONSM的放疗方式有很多,包括空间分割放射治疗、调强放射治疗、立体定向放疗、质子束治疗、三维适形放疗等。手术治疗会导致患者视力丧失,通常不予采用,但新的手术入路,可以防止肿瘤延伸至对侧。药物治疗在一些病例中取得良好的视力改善结果,有望成为治疗ONSM的潜在方法。ONSM死亡率几乎为零,但视力预后通常较差。

Optic nerve sheath meningioma(ONSM)is a rare and slow-growing benign tumor that arises from arachnoid epithelial cells.ONSM primarily affects middle-aged women,and its clinical manifestations are mainly painless-progressive vision loss.Diagnosis is largely based on the combination of clinical and imaging findings.The typical CT/MRI findings are tubular or fusiform thickening of the optic nerve,followed by a“double-track sign”after enhanced scan,which also may be accompanied by calcification.Treatment includes observation,radiotherapy and surgery,among which radiotherapy is the first choice for the treatment of ONSM.At present,there are many radiotherapy methods for ONSM,including spatially fractionated radiation therapy(SFRT),intensity modulated radiation therapy(IMRT),stereotactic radiosurgery(SRS),proton beam radiotherapy(PBT),3-dimensional conformal radiation therapy(3D-CRT),etc.Surgical treatment can cause vision loss in patients and is usually not be used,but new surgical approaches have been proposed in the literature to prevent tumor extension to the contralateral side.Drug therapy has yielded vision improvement in some cases and is expected to be a potential treatment for ONSM.The mortality of ONSM is almost zero,but the visual prognosis is generally poor.