摘要目的 分析乳头状胶质神经元肿瘤(PGNTs)的临床、影像、病理特点和预后.方法 回顾性分析2006年1月至2013年1月首都医科大学附属北京天坛医院神经外科收治的16例PGNTs的临床资料,并进行文献回顾.结果 16例PGNTs中,男9例,女7例,平均年龄为24岁.术前平均远期生活质量评估(KPS)评分为86.3分.额叶(4/16)为好发部位,其次为颞叶、侧脑室和顶枕叶,13例表现为囊性病灶伴或不伴实性成分.全切除12例,次全切2例,部分切除1例,内镜下活检1例.术后平均KPS评分为85.6分.8例Ki-67＜ 1％,其他8例为1％～13％不等.术后3例非全切者和1例全切除者行辅助放疗.平均随访56.2个月,随访KPS评分为92.5分,无复发.文献报道77例(男40例,51.9％),平均年龄为27岁,其中49例Ki-67/MIB-1染色阳性,28例(57.1％)≥1％;平均随访28.0个月,复发6例(10.5％),全切除者和未全切者的复发率分别为5.1％和33.3％.结论 PGNTs为中枢神经系统少见肿瘤,好发于额叶及年轻患者,无性别差异,若无病理证据则较难实现术前确诊.肿瘤全切除者预后较好.虽然该肿瘤多呈惰性特点,但亦具非典型表现.

abstractsObjective To analyze the clinical,radiological and pathological features of papillary glioneuronal tumors (PGNTs).Methods The clinical charts and radiographs of 16 cases of PGNTs in Beijing Tiantan Hospital,Capital Medical University between January 2006 and January 2013 were retrospectively reviewed.The follow-up evaluations and literature review were performed.Results The study included 9 males and 7 females with a mean age of 24 years.Upon admission,the mean preoperative KPS(Karnofsky performance scale)score was 86.3.The radiological results showed that the frontal lobe (4/16) was the most common portion of the brain involved,followed by temporal lobe,lateral ventricle and parieto-occipital lobe,13 lesions presented with cystic appearance with or without solid elements.Complete resection,subtotal resection,partial resection were achieved in 12,2 and 1 patients,respectively.One patient underwent biopsy under neuroendoscope.The mean postoperative KPS score was 85.6.Ki-67 staining was positive in ＜ 1％ of cells present in 8 lesions and varied in the other 8 lesions,with a range of 1％ to 13％.During the follow-up period,3 patients with incomplete removal and 1 with complete resection received radiotherapy.The mean follow-up duration was 56.2 months,the most recent mean KPS score was 92.5,and there was no recurrence.Conclusions PGNTs was a rare central nervous system lesion and was more frequently observed in the frontal lobe and in young patients without sex predilection.The preoperative diagnosis was difficult without pathological evidence.Fair outcomes could be achieved by complete resection.Although PGNTs displayed indolent pathobiology,atypical appearances were observed.