Optic Pathway Gliomas in Adults

被引:16
作者
Shofty, Ben [1 ,2 ]
Constantini, Shlomi [1 ,2 ]
Bokstein, Felix [3 ]
Ram, Zvi [1 ]
Ben-Sira, Liat [4 ]
Freedman, Sigal [1 ]
Vainer, Gilad [5 ]
Kesler, Anat [6 ]
机构
[1] Tel Aviv Med Ctr & Sch Med, Div Neurosurg, IL-64239 Tel Aviv, Israel
[2] Tel Aviv Med Ctr & Sch Med, Gilbert Israeli Neurofibromatosis Ctr, IL-64239 Tel Aviv, Israel
[3] Tel Aviv Med Ctr & Sch Med, Neurooncol Serv, IL-64239 Tel Aviv, Israel
[4] Tel Aviv Med Ctr & Sch Med, Pediat Radiol Unit, IL-64239 Tel Aviv, Israel
[5] Tel Aviv Med Ctr & Sch Med, IL-64239 Tel Aviv, Israel
[6] Tel Aviv Med Ctr & Sch Med, Neuroophthalmol Unit, IL-64239 Tel Aviv, Israel
关键词
Low-grade glioma; Neurofibromatosis; NF1; OPG; Optic pathway gliomas; IDH1; MUTATIONS; MALIGNANT-TRANSFORMATION; RESPONSE CRITERIA; FOLLOW-UP; CHILDREN; TUMORS; SEGMENTATION; COMMON;
D O I
10.1227/NEU.0000000000000257
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
BACKGROUND: Optic pathway gliomas (OPGs) are considered relatively benign pediatric tumors. Adult patients with OPG can be divided into 2 groups: adult patients with tumors diagnosed in childhood and adult patients diagnosed during adulthood. OBJECTIVE: To characterize the clinical course of adult patients with OPG. METHODS: We retrospectively collected clinical and imaging data of all adult OPG patients monitored in our medical center between 1990 and 2012. RESULTS: Twenty-two adult patients were included. Age at diagnosis varied widely (6 months-66 years), as did age at last follow-up (18-74 years). Ten patients were diagnosed at adulthood and 12 in childhood. Of the patients diagnosed at childhood, 6 had radiological progression during childhood, and 3 of those patients suffered visual impairment. From this group, 1 patient had further progression during adulthood accompanied by additional visual decline, and 2 patients had additional visual decline during adulthood despite no signs of progression. Of the 6 patients whose tumors were stable during childhood, all 6 remained stable during adulthood. Of 10 patients diagnosed at adulthood, 6 patients suffered visual deterioration; in 5 of them, a concomitant progression was noted. Two patients were diagnosed with high-grade gliomas. CONCLUSION: OPGs may be active during childhood or adulthood. Those patients who experienced anatomic activity during childhood are prone to continue experiencing active disease during adulthood. A significant percentage of patients diagnosed with low-grade OPG at adulthood may suffer progression, visual decline, or both.
引用
收藏
页码:273 / 279
页数:7
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