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中樞神經(jīng)系統(tǒng)中具有脊索樣特征腫瘤的臨床病理分析

發(fā)布時(shí)間:2018-05-01 00:24

  本文選題:脊索瘤 + 脊索樣腦膜瘤; 參考:《臨床與實(shí)驗(yàn)病理學(xué)雜志》2017年11期


【摘要】:目的探討中樞神經(jīng)系統(tǒng)中具有脊索樣特征腫瘤的臨床病理特征、診斷及鑒別診斷。方法回顧性分析81例脊索瘤、9例脊索樣腦膜瘤、4例脊索樣膠質(zhì)瘤及2例上皮樣血管內(nèi)皮瘤的臨床資料、組織學(xué)特征,并復(fù)習(xí)相關(guān)文獻(xiàn)。結(jié)果脊索樣特征腫瘤鏡下瘤細(xì)胞呈條索狀或巢狀分布于黏液樣間質(zhì)中。脊索瘤好發(fā)于人體中軸,多見(jiàn)于顱底及骶尾部,鏡下呈分葉狀結(jié)構(gòu),可見(jiàn)胞質(zhì)含空泡的液滴細(xì)胞及星芒狀細(xì)胞。免疫表型:CK、EMA、vimentin均陽(yáng)性,絕大多數(shù)S-100陽(yáng)性,GFAP均陰性,PAS染色陽(yáng)性。脊索樣腦膜瘤好發(fā)于幕上,與腦膜組織關(guān)系密切,鏡下可見(jiàn)脊索樣成分與典型腦膜瘤結(jié)構(gòu)混雜,間質(zhì)炎性細(xì)胞浸潤(rùn),可見(jiàn)淋巴濾泡。免疫表型:vimentin、EMA、PR均陽(yáng)性。脊索樣膠質(zhì)瘤好發(fā)于第三腦室,女性多見(jiàn),鏡下可見(jiàn)部分瘤細(xì)胞膠質(zhì)細(xì)胞分化,膠質(zhì)原纖維突起,核分裂象罕見(jiàn),間質(zhì)大量淋巴細(xì)胞、漿細(xì)胞浸潤(rùn)但不形成淋巴濾泡,可見(jiàn)Russell小體,瘤周組織星形細(xì)胞反應(yīng)性增生。免疫表型:GFAP、vimentin均陽(yáng)性,CK陰性。上皮樣血管內(nèi)皮瘤具有上皮樣細(xì)胞和血管內(nèi)皮細(xì)胞的組織學(xué)特征,鏡下見(jiàn)卵圓形或多角形瘤細(xì)胞形成原始血管腔,內(nèi)含紅細(xì)胞,腫瘤細(xì)胞輕度異型性,電鏡下可見(jiàn)W-P小體。免疫表型:CD31、CD34、vimentin均陽(yáng)性。結(jié)論脊索瘤、脊索樣腦膜瘤、脊索樣膠質(zhì)瘤及上皮樣血管內(nèi)皮瘤具有共同的脊索樣特征,發(fā)生于中樞神經(jīng)系統(tǒng)時(shí),其鑒別診斷主要依據(jù)臨床、形態(tài)學(xué)特征及免疫表型綜合分析。
[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.
【作者單位】: 貴州省銅仁市人民醫(yī)院病理科;中南大學(xué)湘雅醫(yī)院病理科/基礎(chǔ)醫(yī)學(xué)院病理學(xué)系;
【分類(lèi)號(hào)】:R739.4
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本文編號(hào):1826910

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