黏液乳头型室管膜瘤10例临床病理分析

Myxopapillary ependymomas:a clinical and pathological analysis of 10 cases

目的探讨黏液乳头型室管膜瘤的临床特点、病理形态、免疫表型及预后。方法分析10例黏液乳头型室管膜瘤的临床病理及预后资料,并进行免疫组化染色。结果患者年龄14～50岁,平均32岁,男女之比为1.5:1。病变多数发生于脊髓圆锥-马尾终丝区。组织学显示肿瘤细胞呈立方形或梭形,以乳头放射状排列在血管间质轴心周围;大量黏液聚集在血管和瘤细胞间或囊腔内;核分裂象很少或缺如。免疫组化示肿瘤细胞GFAP、vimentin和S-100(+),CK和EMA(-)。3例复发者均因首次手术未能完全切除肿瘤所致;2例肿瘤完全切除者均无复发。结论黏液乳头型室管膜瘤几乎只发生在脊髓圆锥及马尾终丝,是室管膜瘤的一种少见亚型,在病理学上具有特征的形态学表现及免疫组化表型。手术治疗是否彻底与预后相关。

Objective To investigate the clinicopathological and ependymomas. Methods Clinicopathological and prognostic features and prognosis of myxopapillary data were analyzed and immunohistochemical staining was performed in 10 cases of myxopapillary ependymoma. Results The mean age of the 10 patients at diagnosis was 32 years （range 14-50 years）. The male to female ratio was 1.5： 1. The locations were almost in the conus tenninalis or ilium terminale. Tumor cells were cubfform or fnsiform, and arranged radically around blood vessels. A large amount of mncoid accumulated between vessels and the tumor cells or within cysts. Mitotic figures were seldomly observed in the tumor cells. Immunohistochemical staining showed that tumor cells were positive for GFAP, vimentin and S- 100, and negative for CK and EMA. Three cases recurred owing to incompleted removal of the tumor in the primary operation. Two cases with grossly total resection of the tumor were relapse-free. Conclusion Myxopapillary ependymoma, a subtype of ependymoma, most frequently arises within conus medullaris, the cauda equina or filum terminale. The prognosis may be related to the extent of resection in patients with myxopapillary ependymoma.