中枢神经系统中具有脊索样特征肿瘤的临床病理分析

发布时间：2018-05-01 00:24

  本文选题：脊索瘤 + 脊索样脑膜瘤　； 参考：《临床与实验病理学杂志》2017年11期

【摘要】：目的探讨中枢神经系统中具有脊索样特征肿瘤的临床病理特征、诊断及鉴别诊断。方法回顾性分析81例脊索瘤、9例脊索样脑膜瘤、4例脊索样胶质瘤及2例上皮样血管内皮瘤的临床资料、组织学特征,并复习相关文献。结果脊索样特征肿瘤镜下瘤细胞呈条索状或巢状分布于黏液样间质中。脊索瘤好发于人体中轴,多见于颅底及骶尾部,镜下呈分叶状结构,可见胞质含空泡的液滴细胞及星芒状细胞。免疫表型:CK、EMA、vimentin均阳性,绝大多数S-100阳性,GFAP均阴性,PAS染色阳性。脊索样脑膜瘤好发于幕上,与脑膜组织关系密切,镜下可见脊索样成分与典型脑膜瘤结构混杂,间质炎性细胞浸润,可见淋巴滤泡。免疫表型:vimentin、EMA、PR均阳性。脊索样胶质瘤好发于第三脑室,女性多见,镜下可见部分瘤细胞胶质细胞分化,胶质原纤维突起,核分裂象罕见,间质大量淋巴细胞、浆细胞浸润但不形成淋巴滤泡,可见Russell小体,瘤周组织星形细胞反应性增生。免疫表型:GFAP、vimentin均阳性,CK阴性。上皮样血管内皮瘤具有上皮样细胞和血管内皮细胞的组织学特征,镜下见卵圆形或多角形瘤细胞形成原始血管腔,内含红细胞,肿瘤细胞轻度异型性,电镜下可见W-P小体。免疫表型:CD31、CD34、vimentin均阳性。结论脊索瘤、脊索样脑膜瘤、脊索样胶质瘤及上皮样血管内皮瘤具有共同的脊索样特征,发生于中枢神经系统时,其鉴别诊断主要依据临床、形态学特征及免疫表型综合分析。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis and differential diagnosis of chordoid tumors in the central nervous system. Methods the clinical data and histological features of 9 cases of chordoid meningioma and 4 cases of chordoid glioma and 2 cases of epithelioid hemangioendothelioma were retrospectively analyzed. Results notochord-like tumor cells were distributed in mucoid stroma under microscope. Chordoma occurs in the central axis of the human body, mostly in the skull base and sacrococcyx. Under the microscope, there are lobular structure, vacuolar droplet cells and stellate cells in the cytoplasm. The immunophenotype was positive for EMA vimentin, and most of S-100 positive for GFAP were negative for pas staining. Spondyloid meningioma usually occurs on the tentorium and is closely related to the meningeal tissue. Under the microscope the chordoid component is mixed with the typical meningioma structure the interstitial inflammatory cells infiltrate and the lymphatic follicles can be seen. The immunophenotype was positive for EMA-PR. Chordoid gliomas are common in the third ventricle, and are more common in women. Some of the tumor cells are found to differentiate into glial cells, glial fibrillar processes, mitosis is rare, interstitial lymphocytes are abundant, plasma cells infiltrate but do not form lymphoid follicles. Russell corpuscles and reactive proliferation of astrocytes around the tumor were found. The immunophenotypes were all positive and CK negative. Epithelioid hemangioendothelioma has the histologic characteristics of epithelioid cells and vascular endothelial cells. Under the microscope, oval or polygonal tumor cells form primitive vascular lumen, contain erythrocytes, and the tumor cells are mild heterotypic. W-P corpuscles can be seen under electron microscope. The immunophenotype of CD31, CD34 and vimentin were all positive. Conclusion chordoma, chordoid meningioma, chordoid glioma and epithelioid hemangioendothelioma have common notochordoid features. The differential diagnosis of chordoma, chordoid meningioma and epithelioid hemangioendothelioma is mainly based on clinical, morphological and immunophenotypic analysis.
【作者单位】： 贵州省铜仁市人民医院病理科;中南大学湘雅医院病理科/基础医学院病理学系;
【分类号】：R739.4
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本文编号：1826910