视神经胶质瘤的临床研究进展

Progress of clinical research on optic pathway glioma

视神经胶质瘤(optic pathwayglioma,OPG)是一种自然病程多变的罕见肿瘤,以儿童多发。OPG与神经纤维瘤病1型(neurofibromatosis type1,NF-1)的关系密切,其中并发NF-1的以前路OPG多见,多侵犯视神经与视交叉,发病年龄较小,常表现为良性自限性肿瘤,症状以视觉损害为主,预后较好;而不并发NF-1的OPG常侵犯视交叉及下丘脑,发病年龄较大,且更具侵袭性,症状除视觉损害外还可表现为内分泌异常,预后相对较差。OPG的病理多为低级别的毛细胞型星形胶质细胞瘤,恶性OPG极为罕见。CT和MRI等影像学检查是诊断和随访OPG的重要手段。对无进展的OPG患者一般只需定期进行眼科及影像学检查随访,进展性OPG可采用手术、放疗、化疗或联合治疗以提高无进展生存率,但关于各种治疗方法的疗效、不良反应和预后对比缺乏大宗病例研究结果,目前尚无公认的最佳方案。

Optic pathway glioma （OPG） is a rare tumer with highly variable natural history, which tends to be present in younger children. OPG has a close relationship with neurofibromatosis type 1 （NF1）. OPGs with NF-1 always involving anterior visual pathway include the optic nerves and chiasm, the age at presentation of which is younger, having a benign self-limiting growth pattern, visual loss as major symptom, and a better prognosis than the others; while those without NF-1 always involve chiasm and hypothalamus, the age at presentation of which is older, manifesting more aggressive, endocrine disorders compound with visual loss as major symptom, and a relatively worse prognosis. The pathology of most OPGs is low-grade pilocytic astrocytomas, and malignant OPGs are rare. Neuroimaging examinations such as CT and MRI are important means to diagnose and follow up OPGs. Generally, OPGs without progression need only routine ophthalmological and neuroimaging examinations, while progressive OPGs need therapeutic options including surgery, radiation, or chemotherapy, or a combination of these modalities to improve the disease-free survival rate. There is still no report of large cohort of patients to compare the outcome, side effect, prognosis of these modalities, and no a widely accepted guideline of management now.