摘要
卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)是一种罕见的具有局部侵袭性的血管肿瘤,好发于婴幼儿,常伴有卡梅现象(Kasabach-Merrittphenomenon,KMP),病情进展快,因此,早期诊断至关重要,对于患者的预后有重要影响。诊断主要包括彩色多普勒超声检查、CT、磁共振及病理检查。超声检查有助于小儿软组织血管异常的诊断、评估病变范围、评估并发症及治疗反应,但由于超声很难清楚地显示KHE的浸润部分,想要通过超声成像准确地评估KHE程度是非常困难的,需要进一步检查。CT有助于检查病灶的骨骼侵袭。核磁共振检查是诊断KHE的重要方式之一,当出现病灶边界不清或浸润性生长血管性肿块伴发KMP,同时伴有斑点状低信号T2加权信号,儿童可明确诊断。KHE组织学特性是血管团由"炮弹状"小叶融合而成,被新月形淋巴管包绕,生物学标志物CD31、CD34、ERG、VEGFR-3、Fli-1常呈阳性表达。而这些特点有助于与其他血管肿瘤进行鉴别。本文就KHE的影像学、病理学诊断等方面的研究进展作一综述。
Kaposiform hemangioendothelioma(KHE)is a rare type of locally invasive vascular tumor that occurs in infants and young children and is often accompanied by Kasabach-Merrittphenomenon(KMP).The disease progresses rapidly.Thus,early diagnosis is crucial and has a significant impact on the prognosis of patients.The diagnosis mainly includes color doppler ultrasound,CT,magnetic resonance and pathological examination.Ultrasound examination is helpful for the diagnosis of soft tissue vascular abnormalities,the assessment of the range of lesions,the assessment of complications and treatment response in children.However,it is difficult to clearly show the infiltrated part of KHE by ultrasound,so it is very difficult to accurately evaluate the degree of KHE by ultrasound imaging,which requires progressive examination.CT scan is helpful in detecting bone invasion.Mri is one of the important ways to diagnose KHE.Children can be clearly diagnosed when there is unclear lesion boundary or invasive growth vascular mass accompanied by KMP accompanied by specular low signal T2 weighted signal.The histological characteristics of KHE are that the vascular mass is formed by the fusion of"cannonball lobules"and is surrounded by lunate-shaped lymphatic vessels.The biological markers CD31,CD34,ERG,vegfr-3 and fli-1 are often expressed positively.These characteristics may help to differentiate them from other vascular tumors.Therefore,this article reviews the research progress of pathological diagnosis and imaging examination of KHE.
作者
苏日太(综述)
杨晓楠(审校)
SU Ri-tai;YANG Xiao-nan(Department of Vascular Surgery,Shunyi Hospital of Bering City,Beijing 101300,China;Department of Plastic Surgery,Chinese Academy of Medical Science,Peking Union Medical School,Plastic Surgery Hospital,Beijing 100144,China)
出处
《中国美容医学》
CAS
2020年第8期186-189,共4页
Chinese Journal of Aesthetic Medicine
