52例脊索瘤临床病理学观察

Clinicopathological observation of 52 cases of chordoma

目的:研究脊索瘤的临床病理特征。方法:对52例脊索瘤患者的临床表现、病理学形态、免疫表型、治疗方法和预后进行回顾性分析。结果:年龄27~79(中位58)岁,其中男29例,女23例。骶尾部46例,蝶鞍部2例,斜坡1例,颈椎2例,腰椎1例。影像学显示溶骨性破坏。形态学瘤细胞呈分叶状结构,富含黏液样基质并可见胞内囊泡,免疫组织化学上皮性标志及S100蛋白阳性表达。1例去分化为纤维肉瘤,且Ki-67增殖指数升高。其中27例(52%)复发,3例转移。结论:脊索瘤多见于中老年人中轴骨,低度恶性,复发常见,可远处转移,病理形态以经典型为主,可发生去分化并提示预后不良。免疫组织化学有助于诊断及鉴别诊断。

Objective： To study the clinicopathologic feature of chordoma. Methods： The clinical data of 52 cases of chordoma were retrospectively analyzed, including clinical features, pathological morphology, immunophenotype,treatment and prognosis. Results： The age ranged from 27–79 years（median age： 58 years）, 29 males and 23 females were included. Forty-six cases occurred in the sacrococcygeal, 2 cases in the sella, 1 case in the slope, 2 cases in the cervical spine and 1 case in the lumbar spine. Radiology images showed the osteolytic destruction.Morphologically, the tumor cells were lobulated, rich in mucin-like matrix and had visible intracellular vesicles.Immunohistochemistry showed epithelial markers and S100 protein were positively expressed. One of these cases dedifferentiated into fibrosarcoma, with an increased Ki67 proliferation index. Among these cases, 27（52%） recurred and 3 metastasized. Conclusion： Chordoma is more common in the axial bone of middle-aged and elderly people, with low degree of malignancy, common recurrence, and possibility of metastasis. The main pathological type is classical type, and sometimes it may occur dedifferentiation suggesting poor prognosis.Immunohistochemistry is contribute to diagnosis and differential diagnosis.