El Beltagy Mohamed A, Reda Mohamed, Enayet Abdelrhman, Zaghloul Mohamed Saad, Awad Madeha, Zekri Wael, Taha Hala, El-Khateeb Nada
Department of Neurosurgery, Kasr Al-Ainy School of Medicine, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
Department of Neurosurgery, Kasr Al-Ainy School of Medicine, Children's Cancer Hospital Egypt, Cairo University, Cairo, Egypt.
World Neurosurg. 2016 May;89:525-34. doi: 10.1016/j.wneu.2016.02.042. Epub 2016 Feb 18.
Optic pathway gliomas (OPGs) are rare neoplasms in children with an unpredictable clinical course. There is significant controversy regarding the optimal management and outcome of these patients.
Charts of all patients with OPG diagnosed and treated at Children's Cancer Hospital Egypt between July 2007 and July 2014 were retrospectively reviewed. We evaluated the roles of surgical, ophthalmologic, endocrinologic, neurologic, and treatment aspects of care.
Sixty-five patients were included in this study, with a mean age of 5.3 years. OPGs were chiasmatic (n = 25), optic nerve (n = 18), hypothalamic (n = 7), and chiasmatic/hypothalamic (n = 7). Extensive involvement of the optic pathway was seen in an additional 8 patients. Twenty cases had neurofibromatosis type 1. Four cases underwent surgical debulking, and 28 were biopsied (16 open, 11 stereotactic, and 1 endoscopic). Nine of the 18 optic nerve tumors were managed by total excision. Twenty-four patients did not undergo any surgical intervention. Forty-five patients received chemotherapy. Histopathology revealed pilocytic (n = 20), pilomyxoid (n = 15), fibrillary astrocytoma (n = 4), and grade I papillary-glioneuronal tumor (n = 1). Nonrepresentative sample (n = 1). The 4-year overall survival rate was 86.3% with mean follow-up period of 32.2 months.
The initial role of surgery in newly developed OPG is biopsy for tissue diagnosis and relief of the hydrocephalus, if present, followed by chemotherapy. Chemotherapy decreases or stabilizes the tumor size in most cases, leading to preservation of both visual and endocrinal functions. The most significant prognostic factor confirmed in this study was the age of the patient.
视路胶质瘤（OPG）是儿童罕见肿瘤，临床病程难以预测。对于这些患者的最佳治疗方案和预后存在重大争议。
回顾性分析2007年7月至2014年7月在埃及儿童癌症医院诊断和治疗的所有OPG患者的病历。我们评估了手术、眼科、内分泌、神经科及治疗方面的护理作用。
本研究纳入65例患者，平均年龄5.3岁。视路胶质瘤位于视交叉（n = 25）、视神经（n = 18）、下丘脑（n = 7）及视交叉/下丘脑（n = 7）。另有8例患者视路广泛受累。20例患有1型神经纤维瘤病。4例接受手术减瘤，28例进行活检（16例开放活检、11例立体定向活检和1例内镜活检）。18例视神经肿瘤中有9例通过全切治疗。24例患者未接受任何手术干预。45例患者接受化疗。组织病理学显示为毛细胞型（n = 20）、毛黏液样型（n = 15）、纤维型星形细胞瘤（n = 4）及I级乳头状胶质神经元肿瘤（n = 1）。非典型样本（n = 1）。4年总生存率为86.3%，平均随访期为32.2个月。
新发生的视路胶质瘤手术的首要作用是活检以进行组织诊断，如有脑积水则予以缓解，随后进行化疗。化疗在大多数情况下可减小或稳定肿瘤大小，从而保留视觉和内分泌功能。本研究中确认的最显著预后因素是患者年龄。
