CAJ | 학술논문

目的探讨婴幼儿卡波西型血管内皮瘤(Kaposiform hemangioendothelioma,KHE)的临床及病理学特征.方法回顾性分析2013年1-12月河南省人民医院诊断10例KHE的临床及病理资料,用免疫组织化学方法检测肿瘤组织中CD34、CD31、GLUT-1、Ki-67、D2-40、SMA、ERG等指标的表达情况.结果本组10例KHE均为婴幼儿,年龄13 d～13个月,肿瘤均发生于浅表皮肤,并累及深部软组织,最大径4 ～ 10 cm,其中8例位于四肢,4例病变皮肤累及范围广,8例合并卡麦综合征.肿瘤组织由增生的梭形和上皮样内皮细胞结节构成,结节周边可见新月形裂隙状血管.肿瘤细胞弥漫表达CD34、CD31、ERG,部分表达D2-40和SMA,总体Ki-67指数低,血管腔内滞留的红细胞表达GLUT-1.10例均行手术切除,1例术后第11天死亡,其余9例均痊愈出院,术后无复发.结论 KHE是一种中间型血管肿瘤,具有局部侵袭性,需要与好发于婴幼儿其他的相似良恶性肿瘤鉴别,卡麦综合征是其常见合并症.早期诊断和治疗能明显提高KHE患儿的生存率.
목적 탐토영유인잡파서형혈관내피류(Kaposiform hemangioendothelioma,KHE)적림상급병이학특정.방법 회고성분석2013년1-12월하남성인민의원진단10례KHE적림상급병리자료,용면역조직화학방법검측종류조직중CD34、CD31、GLUT-1、Ki-67、D2-40、SMA、ERG등지표적표체정황.결과 본조10례KHE균위영유인,년령13 d～13개월,종류균발생우천표피부,병루급심부연조직,최대경4 ～ 10 cm,기중8례위우사지,4례병변피부루급범위엄,8례합병잡맥종합정.종류조직유증생적사형화상피양내피세포결절구성,결절주변가견신월형렬극상혈관.종류세포미만표체CD34、CD31、ERG,부분표체D2-40화SMA,총체Ki-67지수저,혈관강내체류적홍세포표체GLUT-1.10례균행수술절제,1례술후제11천사망,기여9례균전유출원,술후무복발.결론 KHE시일충중간형혈관종류,구유국부침습성,수요여호발우영유인기타적상사량악성종류감별,잡맥종합정시기상견합병증.조기진단화치료능명현제고KHE환인적생존솔.
Objective To discuss the clinical and pathological features of Kaposiform hemangioendothelioma (KHE).Methods 10 cases of KHE treated in our hospital were analyzed restrospectively in 2013,and immunohistochemistry was used to detect the expression of CD34,CD31,GLUT-1,Ki-67,D2-40,SMA,ERG in tumor tissues.Results The KHE 10 cases were infants and neonates aged between 13 days to 13 months.Tumors presented as a superficial cutaneous and soft tissue mass with the maximum diameter ranging from 4 cm to 10 cm.Tumors developed in the extremities in 8 cases.Skin was extensively involved in 4 cases.8 cases were complicated with Kasabach-Merritt syndrome (KMS).The pathological characteristics of KHE are fascicles of spindle shaped endothelial cells and slitlike vascular channels with irregular tumor margins and rare mitoses.Tumor cells diffusely expressed CD34,CD31 and ERG,and partly expressed D2-40 and SMA with a low Ki-67 labeling index.Erythrocytes in the channels were numerous GLUT-1 positive.All cases underwent tumor excision,9 cases were cured and one died.Conclusions KHE is presently classified as a vascular neoplasm of intermediate malignant potential with local invasion,differential diagnosis from other benign and malignant tumors common in infants is necessary;KHE occurs in association with the KMS.Early diagnosis and treatment prolongs the survival.