颅内硬膜下脊索瘤的临床病理特点

Clinical and pathological features of intradural retroclival chordoma

目的探讨原发颅内硬膜下脊索瘤的临床特征及病理特点，以提高对该病的认识。方法对2006年1月至2011年6月经手术、病理及影像证实的6例颅内硬膜下脊索瘤患者进行回顾性研究。其中男5例，女1例，中位发病年龄为40．5岁，多以头痛、复视作为首发症状。病变均位于斜坡硬膜下桥前池内，CT显示颅底骨质无破坏，MRI表现多样。采用SP法对6例标本的石蜡切片行Brachyury、Galectin-3、Ki-67免疫组化检测。根据病变部位采用颞枕、远外侧或乙状窦前入路病变切除术。结果3例全切除，3例近全切除。6例病理均为经典型脊索瘤，Brachyury、Galectin-3染色均为强阳性，Ki-67核阳性指数在2．5％～8．2％。平均随访58．7个月，3例未见复发或再生长，3例肿瘤复发或再生长，其中2例已死于该病。结论颅内硬膜下脊索瘤多见于中年人，临床表现以头痛、脑神经麻痹及锥体束征为主。开颅手术应力争全切除肿瘤。Brachyury、Galectin-3和Ki-67染色阳性可与良性脊索瘤、脊索样脑膜瘤鉴别。预后呈两极分化，肿瘤供血丰富，瘤细胞呈“铺片状”排列，Ki-67指数高于5％者易短期复发，预后差。

Objective To investigate the clinical and characteristics of intracranial intradural chordoma. Method Six cases of intradural retroclival chordomas were operated in our hospital between Jan. 2006 and Jun. 2011. The clinical characteristics and outcome of them were observed retrospectively. There were 5 male and 1 female patients. The mean age of presentation was dO. 5 years. Headache and diplopia were the first clinical symptoms. The preoperative imagings varied according to each patienL Inanunohistochemical staining for Brachyury, Galectin - 3 and Ki -67 was performed in all of them Results All the six lesions were resected by subtemporal, far--lateral or retrosignoid approach, and 3 lesions were totally removed with the rest being subtotally resected. Immunohistochemical patterns showed that all cases were strong positive for Brachyury and Galectin - 3. Ki -67 labeling index was between 2. 5% and 8. 2%. Mean following up period was 58. 7 months. 3 cases presented no signs of recurrence or regrowth, while the other 2 cases died of this disease. One patient received two more operations after discharged because of tumor regrowth. Condusiolas Intradural retroclival chordorms are rare lesions eceuring in middle - aged adults. Headache and paralysis of cranial nerves are the mainly clinical presentation. Totally resection by craniotomy is recommended, which achieved 50% in this series. Posivite stain for Brachyury, Galectin - 3 and Ki-67 is helpful for differential diagnosis with Ecchordosis physaliphora and chordoid meningionm. Its prognosis demonstrated significant differences. Tufters with rich blood supply, creeping cellular arrangement and Ki -67 labeling index above 5% ,are prone to short -term recurrence and poorer prognosis.