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目的探讨鼻腔鼻窦型血管外皮细胞瘤(SNTHPC)的临床病理特征、免疫表型和鉴别诊断。方法回顾性分析5例SNTHPC的临床资料、组织学形态和免疫组化标记,并以6例软组织孤立性纤维性肿瘤(SFT)作为观察对照。结果 5例SNTHPC中女性4例,男性1例,发病平均年龄54岁;发生于左鼻腔2例,右鼻腔3例。术前症状主要为鼻塞和鼻出血。肿块平均直径2.6 cm。镜下见病变位于黏膜下,瘤细胞密度较大,呈短束状、席纹状或旋涡状排列,其中2例可见细胞致密区和疏松区交替分布的现象。细胞间有较多大小不等的薄壁血管,管腔呈圆形、不规则形或裂隙状,瘤细胞围绕血管生长呈血管外皮瘤样。2例可见少数血管壁玻璃样变性。瘤细胞呈圆形、卵圆形或梭形,胞质轻度嗜酸性,细胞界限清楚,核轻度异型性,核分裂象1～3个/10HPF,不见非典型核分裂象。均可见细胞间红细胞外渗,3例可见出血;均可见散在分布的肥大细胞、嗜酸性粒细胞和淋巴细胞浸润,但均未见坏死。免疫组化示瘤细胞vimentin和SMA(+),血管D2-40均(+)。6例SFT vimentin、CD34、CD99和bcl-2(+),2例SMA呈灶性弱(+)。术后随访0.5～2.5年,1例复发。结论 SNTHPC是一种好发于成人鼻腔和鼻窦的少见软组织肿瘤,可局部复发但罕见转移。
Objective To investigate the clinicopathological features, immunophenotypes and differential diagnosis of nasal sinusoidal hemangiopericytoma (SNTHPC). Methods The clinical data, histological features and immunohistochemical markers of 5 cases of SNTHPC were retrospectively analyzed. Six cases of soft tissue solitary fibrous tumor (SFT) were taken as the control. Results In 5 cases of SNTHPC, there were 4 females and 1 males. The average age of onset was 54 years old. There were 2 cases in the left nasal cavity and 3 cases in the right nasal cavity. Preoperative symptoms are mainly nasal congestion and nose bleeding. Mass average diameter of 2.6 cm. Microscopically, the lesion was located under the mucous membrane. The tumor cells were dense, with short bundles and stripes or swirling arrangement. In two cases, the dense and loose cell areas were alternately distributed. Cells between the more sizes ranging from thin-walled vessels, the lumen was round, irregular or slit-like, tumor cells around the blood vessels showed vascular endothelial tumor-like. 2 cases showed a few vascular wall degeneration. Tumor cells were round, oval or fusiform, slightly eosinophilic cytoplasm, clear cell boundaries, mild nuclear atypia, mitotic figures 1 ~ 3 / 10HPF, no atypical mitosis. All of them showed extracellular erythrocyte extravasation and three cases showed hemorrhage. All showed the infiltration of scattered mast cells, eosinophils and lymphocytes, but none of them showed necrosis. Immunohistochemistry showed tumor cells vimentin and SMA (+), vascular D2-40 (+). 6 cases of SFT vimentin, CD34, CD99 and bcl-2 (+), 2 cases of SMA was focal weak (+). Follow-up 0.5 to 2.5 years after operation and 1 case recurred. Conclusion SNTHPC is a rare soft tissue tumor in adult nasal cavity and paranasal sinus. It can be locally recurrent but rarely metastasize.