卡波西样血管内皮瘤的声像图特征及病理学表现  

The ultrasonographic features and pathological manifestations of Kaposiform hemangioendothelioma

作　　者：刘祎斐 袁建军 申凯凯 郭玉娟 李闯 吴刚 LIU Yifei;YUAN Jianjun;SHEN Kaikai;GUO Yujuan;LI Chuang;WU Gang(Department of Ultrasound,Peoples Hsopital of Zhangzhou University,Henan Provincial Peoples Hospital,Zhengzhou 450003,China)

机构地区：[1]郑州大学人民医院河南省人民医院超声科,河南郑州450003

出　　处：《医学影像学杂志》2023年第5期862-865,共4页Journal of Medical Imaging

摘　　要：目的探讨卡波西样血管内皮瘤(KHE)的声像图特征及病理学表现。方法选取我院收治的经术后病理证实的30例KHE患儿的超声检查资料及病理结果,其中18例临床实验室检查及体格检查符合KHE伴有卡梅现象(KMP)。30例患儿均为体表单发病灶伴皮肤或皮下边界不清的红褐色或紫色斑块。所有病灶术前均行超声检查,观察病灶发生部位,总结病灶声像图特征,包括大小、回声、边界、形态、血供情况,对比分析单纯KHE组与KHE伴KMP组的声像图特征。病灶经手术切除后送病理学检查,总结病理组织学及免疫组化表现。结果30例KHE病灶中12例位于头颈部,10例位于四肢,8例位于躯干;19例仅累及皮下组织,11例累及皮下组织及周围肌层;病灶直径范围约12.0~136.0 mm(平均直径58.0 mm),二维声像图均表现为实性不均质回声,其中27例以低回声为主,3例以高回声为主,27例内部未显示明显管样无回声区,3例内部显示管样无回声区;边界不清晰伴周围组织增厚回声增强者29例,边界清晰者1例。彩色多普勒声像图表现中29例内部显示丰富血流信号呈树枝状分布,1例内部未见明显血流信号。KHE伴KMP组较单纯KHE组病灶直径更大,累及部位更多、范围更广。30例病灶发生部位不同,镜下表现大致相同,可见交叉梭形细胞束,呈弥漫性多结节状浸润性生长,结节之间为多少不等的纤维间隔组织,29例核分裂象少见,1例核分裂象多见。免疫组化中肿瘤细胞VEGFR-3、ERG、CD31、CD34、CD117、DOG-1均呈阳性表达,GLUT-1和HHV8均呈阴性表达,Factor VIII呈阴性或局灶性表达,Ki67增殖指数3%~40%(平均8%),部分病例D2-40、FLI-1呈阳性表达,EMA呈阴性表达。结论KHE临床较为罕见,部分可伴有KMP现象。KHE的病理学表现及声像图表现具有显著特异性,超声检查可作为首选检查,为临床明确诊断指导治疗提供可靠依据。Objective To investigate the ultrasonographic features and pathologicalmanifestations of Kaposiform hemangioendothelioma(KHE). Methods The pathological results and ultrasonographic data of 30 KHE children visited Hemangioma Surgical Department of Henan Provincial People's Hospital were retrospectively analyzed. The KHEs in 30 cases were confirmed by postoperative pathology, and the clinical laboratory and physical examinations of 18 cases were consistent with KHE with Kasabach-Merritt Phenomenon(KMP). The 30 cases all revealed body surface single lesions with reddish-brown or purple plaques with obscure skin or subcutaneous boundaries. All lesions were examined by ultrasound before surgery to observe the locations of the lesions and to summarize the ultrasonographic features of the lesions, including size, echo, boundary, shape and blood supply. The ultrasonographic features of the KHE group and the KHE with KMP group were analyzed comparatively. The lesions were surgically removed for pathological examination to summarize histopathological and immunohistochemical manifestations. Results Among 30 KHE lesions, 12 were in the head and neck, 10 were in the extremities, and 8 were in the trunk;19 only involved subcutaneous tissue, and 11 involved subcutaneous tissue and surrounding muscle layer;and the diameter of the lesions was about 12.0~136.0 mm(average 58.0 mm). The two-dimensional ultrasonograms all showed solid heterogeneous echoes, of which 27 cases showed mainly low echoes, and 3 cases showed mainly high echoes;there was no obvious tube-like anechoic area inside 27 cases, and 3 cases showed tube-like anechoic areas inside;29 cases peresented showed obscure boundaries with thickened surrounding tissues and enhanced echoes, 1 case showed clear boundary. In the color Doppler ultrasonograms, 29 cases showed abundant blood flow signals in a dendritic distribution, and no obvious blood flow signal was observed in 1 case. Compared with the KHE group,the KHE with KMP group had larger lesion diameters,more involved p

关 键 词：卡波西样血管内皮瘤 卡梅综合征 病理学 超声检查 

分 类 号：R738.6[医药卫生—肿瘤] R445.1[医药卫生—临床医学]