摘要目的 探讨广泛黏液样和玻璃样变性的鼻腔鼻窦毛细血管瘤(EMH?SNCH)的临床病理学特征.方法 收集2014年1月至2017年12月浙江大学医学院附属第一医院和浙江省人民医院诊治的EMH?SNCH共3例,观察其临床特征、组织学形态、免疫表型特点并结合文献进行复习和总结.结果 3例患者均为老年男性,年龄分别为65、51和80岁.临床上均表现为鼻塞伴反复出血.肿瘤最大径分别为2.0、3.5和1.8 cm.镜下病变由随意分布的弥漫增生的毛细血管样血管组成,分布于广泛黏液样和玻璃样变性的疏松间质中,黏液样和玻璃样变区域占据肿瘤的绝大部分,比例80%~95%.病变内均可见弥漫散在分布于肿瘤内的鹿角形分支状血管以及扩张的血管伴血栓形成,在病变的深部可见局灶富于细胞、呈小叶状排列的毛细血管簇.免疫组织化学染色结果,血管内皮细胞弥漫表达CD31、CD34和ERG,膜表达β?catenin,血管腔周围的肌周皮细胞表达平滑肌肌动蛋白.3例患者术后随访16~46个月,均未见肿瘤复发.结论 EMH?SNCH是鼻腔鼻窦一种少见组织学类型的毛细血管瘤,生物学行为惰性,其形态学鉴别诊断谱系宽广,仔细的形态学观察辅以免疫组织化学染色可助于其诊断和鉴别诊断.

abstractsObjective To investigate the clinicopathological features of extensively myxoid and hyalinized sinonasal capillary hemangioma (EMH?SNCH). Methods Three cases of EMH?SNCH were collected from the First Affiliated Hospital of Zhejiang University School of Medicine and Zhejiang Provincial People′s Hospital from January 2014 to December 2017. The clinical features, histomorphologic changes and immunohistochemical findings were analyzed and the relevant literature was reviewed. Results All three patients were elderly men aged 65, 51 and 80 years, respectively. Clinically, all cases presented with nasal congestion with repeated epistasis. The maximum diameters of the tumors were 2.0, 3.5 and 1.8 cm respectively. Microscopically, the lesions consisted of randomly distributed, diffusely proliferating capillary?like blood vessels within a loose interstitium with extensive myxoid and hyalinized degeneration. Myxoid and hyalinized regions occupied the vast majority of tumors, ranging from 80% to 95%. Scattered staghorn?shaped branching blood vessels were seen in the tumor, some of which were distended by thrombi. In the deep part of the lesion, capillary clusters rich in endothelial cells with focal lobular arrangement were observed. Immunohistochemically, the vascular endothelial cells expressed CD31, CD34 and ERG diffusely and β?catenin at the membrane, and the pericytes surrounding the capillaries expressed SMA. All three patients were followed up for 16 to 46 months, and no tumor recurrence was observed. Conclusions EMH?SNCH is a rare subtype of capillary hemangioma of the sinonasal cavity. It is biologically benign and its histological differential diagnosis spectrum is broad. Careful morphologic observation combined with immunohistochemical staining can help its diagnosis and differential diagnosis.