Lena G, Pech-Gourg G, Scavarda D, Klein O, Paz-Paredes A
Service de neurochirurgie pédiatrique, hôpital des Enfants-La Timone, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France.
Neurochirurgie. 2010 Apr-Jun;56(2-3):249-56. doi: 10.1016/j.neuchi.2010.02.015. Epub 2010 Mar 19.
Optic pathway gliomas are rare tumors accounting for 3-5% of brain tumors in children; 90% are observed in children. They account for 4% of orbital tumors in children. Association with NF 1 is classical and the incidence of NF 1 in patients with optic pathway gliomas is estimated at 30-58%. Usually these tumors are pilocytic astrocytomas and their regression, particularly in patients with NF 1, has been described. Exophthalmos and loss of visual acuity or blindness are the usualpresentation in progressive disease. CT and above all MRI study of the orbit and the brain are necessary to make a complete evaluation of the disease. Gross total removal of the tumor provides 100% cure, but surgery can be proposed only for growing tumor with severe exophthalmos and no useful field of view or blindness. Chemotherapy must be proposed as first-line treatment for growing tumor with moderate exophthalmos and useful vision when the tumor is strictly confined in the orbit. The place of radiotherapy for pure intraorbital gliomas has not been defined and proton beam therapy has to be evaluated.
视神经通路胶质瘤是罕见肿瘤，占儿童脑肿瘤的3% - 5%；90%在儿童中被观察到。它们占儿童眼眶肿瘤的4%。与神经纤维瘤病1型（NF 1）相关是典型情况，视神经通路胶质瘤患者中NF 1的发病率估计为30% - 58%。通常这些肿瘤是毛细胞型星形细胞瘤，并且其消退情况，特别是在NF 1患者中，已有描述。眼球突出以及视力丧失或失明是进展性疾病的常见表现。眼眶和脑部的CT尤其是MRI检查对于全面评估该疾病是必要的。肿瘤全切可实现100%治愈，但仅对于伴有严重眼球突出且无有用视野或失明的生长性肿瘤才可考虑手术。对于伴有中度眼球突出且视力尚好、肿瘤严格局限于眼眶内的生长性肿瘤，必须将化疗作为一线治疗方案。单纯眶内胶质瘤的放疗地位尚未明确，质子束治疗有待评估。
