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目的探讨鼻腔鼻窦型血管外皮瘤的临床病理学特征及诊断和鉴别诊断及其组织发生。方法结合其临床和影像学特点,应用HE、嗜银网状纤维染色、免疫组织化学和电镜对6例鼻腔鼻窦型血管外皮瘤的病理改变进行了分析,并对其中5例进行了随访。结果男性4例,女性2例,年龄56~71岁,平均60·5岁;肿瘤均位于右鼻腔,分别为鼻中隔1例、中鼻道1例、右总道1例、鼻腔+筛窦2例、右眼球+筛窦+左右额窦1例,临床上表现为鼻塞、鼻出血、鼻腔分泌物增多及眼球胀痛、视力减退、流泪、头痛等症状。巨检:病变呈息肉样2例、呈棕色光滑肿块3例、呈灰白碎组织1例。镜下观察呈弥漫分布的短梭形细胞,规则,核分裂象少见,胞质透明或嗜酸性,血管丰富,薄壁,并可见管壁玻璃样变,鹿角状血管少见,瘤细胞多以单相排列,也可呈束状、席纹状、环状及编织状。免疫组织化学检测显示瘤细胞波形蛋白6/6阳性、平滑肌肌动蛋白5/6阳性和CD343/6阳性,超微结构显示瘤细胞胞质内有肌动蛋白丝,细胞间有未发育完全的连接结构。随访5例,2例复发。结论鼻腔鼻窦型血管外皮瘤是一种真正起源自血管外皮细胞,具有特殊临床病理特征、少见的中间型或低度恶性肿瘤,需结合肿瘤的组织形态和免疫组织化学特点做出诊断和鉴别诊断。
Objective To investigate the clinicopathological characteristics, diagnosis, differential diagnosis and histogenesis of nasal sinus type hemangiopericytoma. Methods According to the clinical and radiological features, the pathological changes of 6 cases of nasal sinusoid hemangiopericytoma were analyzed by HE staining, silver staining, immunohistochemistry and electron microscopy, and 5 of them were followed up. Results There were 4 males and 2 females, aged from 56 to 71 years (average 60.5 years). The tumors were located in the right nasal cavity, one in nasal septum, one in midbrain, one in right total tract, one in nasal cavity and ethmoid sinus Cases, right eye + ethmoid + frontal sinus in 1 case, clinically manifested as stuffy nose, nose bleeding, nasal secretions and eye pain, vision loss, tearing, headache and other symptoms. Giant examination: lesions were polypoid in 2 cases, 3 cases were smooth brown, 1 case of gray broken tissue. Microscopic observation of diffusely distributed short spindle cells, regular mitosis rare, cytoplasm transparent or eosinophilic, rich in blood vessels, thin-walled, and visible glass wall-like changes, rare antler-like blood vessels, mostly single-phase tumor cells Arrangement, but also was a beam, Xi Wen-shaped, circular and weaving. Immunohistochemistry showed that the tumor cells were positive for vimentin 6/6, positive for smooth muscle actin 5/6 and positive for CD343 / 6. The ultrastructure showed that there were actin filaments in the cytoplasm of the tumor cells with undeveloped cells Connection structure. Follow-up in 5 cases, 2 cases of recurrence. Conclusions Nasal and sinusoidal hemangiopericytoma is a type of neoplasm derived from vascular epithelial cells with special clinicopathological features and rare intermediate or low grade malignant tumors. Diagnosis and differential diagnosis should be made according to the histological features and immunohistochemical features of the tumor .