摘要
目的 探讨Kaposi型血管内皮瘤的临床病理特征,为临床诊治提供参考依据。方法 回顾2014—2020年广东省妇幼保健院确诊的3例Kaposi型血管内皮瘤的临床和病理资料,并进行组织学和免疫组织化学分析。结果 患者均为婴幼儿,年龄分别为29天、1个月11天和3个月,病例1位于胸腹部,病例2位于左膝关节外侧,病例3位于左侧季肋部,均发生于皮肤或皮下浅表软组织,肉眼观肿瘤呈紫红色,实性,质韧,边界欠清。镜下肿瘤呈多结节状,结节由短梭形血管内皮细胞及血管周细胞组成,簇状分布,形成肾小球样结构。免疫组化CD31、CD34、FⅧ、ERG、D2-40、SMA表达阳性,Glut-1表达阴性。1例近期随访无复发,2例失访。结论 Kaposi型血管内皮瘤是一种好发于婴幼儿的罕见的血管源性肿瘤,主要病理特征是异常的血管生成和淋巴管生成,临床上可伴发不同程度的凝血功能异常,应注意与不同类型的血管肿瘤相鉴别。
Objective To explore the clinicopathological characteristics of Kaposiform hemangioendothelioma, and to provide reference for clinical diagnosis and treatment. Methods The clinical and pathological data of 3 cases of Kaposiform hemangioendothelioma diagnosed in our hospital from 2014 to 2020 were reviewed, and histological and immunohistochemical analyses were performed. Results The patients were all infants. The ages were 29 days, 1 month and 11 days, and 3 months, respectively. Case 1 was located in the chest and abdomen, case 2 on the outside of the left knee joint, case 3 on the left side of the ribs, which occurred in the skin or subcutaneous superficial soft tissues. Macroscopically, the tumors were purple-red, solid, tough, and unclear. Under the microscope, the tumor was multi-nodular, and the nodules were composed of short spindle-shaped vascular endothelial cells and perivascular cells, which distributed in clusters and formed a glomerular-like structure. Immunohistochemistry showed positive expression of CD31, CD34, Fli-1, FⅧ, ERG, D2-40 and SMA, and negative expression of Glut-1. One case had no recurrence in recent follow-up. Two cases were lost to follow-up. Conclusion Kaposiform hemangioendothelioma is a rare angiogenic tumor that tends to occur in infants and young children. The main pathological features are abnormal angiogenesis and lymphangiogenesis. It could be accompanied by different degrees of abnormal blood coagulation in clinical practice, and attention should be paid to distinguish different types of vascular tumors.
作者
杜瑞
吴坤河
彭海艳
吕威
李辉斌
万志彬
张姗姗
秦艳
郜红艺
DU Rui;WU Kun-he;PENG Hai-yan;LV Wei;LI Hui-bin;WAN Zhi-bin;ZHANG Shan-shan;QIN Yan;GAO Hong-yi(Department of Pathology y Guangdong Women and Children Hospital,Guangdong,Guangzhou 511400,China)
出处
《诊断病理学杂志》
2022年第4期326-330,335,共6页
Chinese Journal of Diagnostic Pathology
