Shofty Ben, Constantini Shlomi, Bokstein Felix, Ram Zvi, Ben-Sira Liat, Freedman Sigal, Vainer Gilad, Kesler Anat
*Division of Neurosurgery, ‡Gilbert Israeli Neurofibromatosis Center, §Neuro-Oncology Service, ¶Pediatric Radiology Unit, ‖Pathology, and #Neuro-Ophthalmology Unit, Tel-Aviv Medical Center, and Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.
Neurosurgery. 2014 Mar;74(3):273-9; discussion 279-80. doi: 10.1227/NEU.0000000000000257.
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.
To characterize the clinical course of adult patients with OPG.
We retrospectively collected clinical and imaging data of all adult OPG patients monitored in our medical center between 1990 and 2012.
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.
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.
NF1, neurofibromatosis 1OPG, optic pathway gliomas.
视路胶质瘤(OPG)被认为是相对良性的儿童肿瘤。成年OPG患者可分为两组:儿童期诊断出肿瘤的成年患者和成年期诊断出的成年患者。
描述成年OPG患者的临床病程。
我们回顾性收集了1990年至2012年间在我们医疗中心接受监测的所有成年OPG患者的临床和影像数据。
纳入了22例成年患者。诊断时的年龄差异很大(6个月至66岁),最后一次随访时的年龄也是如此(18至74岁)。10例患者在成年期被诊断出,12例在儿童期被诊断出。在儿童期被诊断出的患者中,6例在儿童期有影像学进展,其中3例患者出现视力损害。在这组患者中,1例在成年期有进一步进展并伴有额外的视力下降,2例患者在成年期出现额外的视力下降,尽管没有进展迹象。在6例肿瘤在儿童期稳定的患者中,所有6例在成年期仍保持稳定。在成年期被诊断出的10例患者中,6例患者出现视力恶化;其中5例伴有肿瘤进展。2例患者被诊断为高级别胶质瘤。
OPG在儿童期或成年期可能处于活跃状态。那些在儿童期经历解剖学活动的患者在成年期容易继续出现疾病活动。相当一部分成年期被诊断为低级别OPG的患者可能会出现进展、视力下降或两者兼有。
NF1,神经纤维瘤病1型;OPG,视路胶质瘤