【摘要】：目的:探討脾竇岸細(xì)胞血管瘤(littoral cell angioma,LCA)的臨床病理特征、形態(tài)學(xué)特點(diǎn)和免疫表型,為正確的診斷、避免誤診提供依據(jù)。方法:回顧性研究13例LCA患者的臨床病理資料、組織學(xué)特點(diǎn)并將LCA的石蠟包埋標(biāo)本進(jìn)行免疫組織化學(xué)染色,同時(shí)將5例海綿狀血管瘤和4例正常脾竇岸細(xì)胞作為對照組。結(jié)果:13例LCA患者包括7名男性和6名女性,年齡從39~70歲,平均54.2歲,中位年齡55歲,這些腫瘤患者中,6例伴有腹腔內(nèi)惡性、良性腫瘤或者炎癥,7例由體格檢查被偶然發(fā)現(xiàn)。腫瘤標(biāo)本大體觀發(fā)現(xiàn)脾內(nèi)含有直徑為0.5~6.2 cm孤立性或者多發(fā)性灰白色結(jié)節(jié)。腫瘤組織學(xué)特征為由相互吻合的血管腔隙構(gòu)成,血管腔隙內(nèi)排列豐富的、從圓形到立方狀竇岸細(xì)胞,并延伸到血管腔,通�？梢钥吹皆谀[瘤的一些區(qū)域中,覆蓋于乳頭狀結(jié)構(gòu)中的竇岸細(xì)胞延伸到血管腔內(nèi),在血管腔中還可以辨認(rèn)出另外一種組織細(xì)胞樣細(xì)胞,大小比竇岸細(xì)胞大,兩種細(xì)胞均未顯示細(xì)胞學(xué)非典型性。免疫組織化學(xué)顯示所有病例中的竇岸細(xì)胞對血管內(nèi)皮細(xì)胞和組織細(xì)胞標(biāo)記物(如CD21、CD31、CD68、多克隆FⅧRAg和ERG)呈陽性表達(dá),而這些細(xì)胞對CD8、CD34和WT-1呈陰性表達(dá),表明LCA中的竇岸細(xì)胞的免疫表型與對照組不同。結(jié)論:LCA是一種良性病變,多發(fā)生在老年人,它的病因尚不清楚,然而,由于在一些病例中伴隨的其他腫瘤或炎癥,因此免疫失調(diào)可能和該腫瘤存在關(guān)聯(lián),LCA中的竇岸細(xì)胞在免疫組織化學(xué)中呈內(nèi)皮細(xì)胞-組織細(xì)胞混合性表型,因此這些細(xì)胞可能具有介于內(nèi)皮細(xì)胞和組織細(xì)胞之間的特征,強(qiáng)調(diào)組織學(xué)檢查和免疫表型對診斷和鑒別診斷至關(guān)重要。
[Abstract]:Objective: to investigate the clinicopathological features, morphological features and immunophenotype of splenic sinus hemangioma (littoral cell) in order to provide a basis for correct diagnosis and avoid misdiagnosis. Methods: the clinicopathological data and histological features of 13 patients with LCA were retrospectively studied. The paraffin embedded specimens of LCA were stained with immunohistochemistry, while 5 cavernous hemangioma and 4 normal splenic sinus bank cells were used as control group. Results of the 13 LCA patients, 7 were male and 6 were female, aged from 3970 years (mean 54.2 years), with a median age of 55 years. 6 of these patients were associated with intraperitoneal malignancy, and 7 patients with benign tumor or inflammation were accidentally found by physical examination. Tumor specimens showed that the spleen contained solitary or multiple grayish white nodules with a diameter of 0.5 ~ 6.2 cm. The histologic features of the tumor are composed of anastomosed vascular lacunae, which are rich in arrangement, ranging from round to cubic sinus shore cells, and extending into the vascular lumen, usually seen in some areas of the tumor. The sinus bank cells covered in the papillary structure extended into the vascular lumen and another histiocyte-like cell was identified in the vascular cavity. The size of the cells was larger than that of the sinus bank cells. Neither of the two cells showed cytological heterogeneity. Immunohistochemical staining showed that the sinusoidal cells in all cases showed positive expression of vascular endothelial cells and histocyte markers (such as CD21, CD31, CD68, polyclonal F 鈪，

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