目的 探讨卡波西型血管内皮瘤（Kaposiform hemangioendothelioma,KHE）的临床病理学特征、诊断及鉴别诊断。方法对29例KHE的组织病理学特征和免疫表型进行观察,结合临床资料进行分析,并复习相关文献。结果 29例KHE中,男性15例,女性14例,除1例患者年龄25岁外,其余均1岁;发生于浅表皮肤28例,包括四肢15例、头颈部5例、躯干4例、腋窝1例、多部位同时累及3例,盆腔内1例。临床表现为局限或弥漫的皮肤无痛性斑块,其中16例伴卡梅现象。镜下肿瘤由增生的小血管和梭形细胞构成,呈不规则结节状或分叶状分布,浸润性生长,梭形细胞呈束状排列,并见上皮样细胞和梭形细胞排列成巢,似肾小球样结构,后者边缘有月牙状血管裂隙形成。免疫表型：瘤细胞VEGFR-3、CD31、CD34、ERG强阳性,部分细胞SMA和D2-40阳性,Glut-1和HHV8恒定阴性,上皮样细胞CK阴性。结论 KHE是一种罕见的好发于婴幼儿和年轻成人的中间型局部侵袭性血管源性肿瘤,诊断主要靠病理组织学及免疫组化标记,须与簇状血管瘤、婴儿血管瘤、先天性血管瘤、梭形细胞血管瘤、疣状静脉畸形和卡波西肉瘤等鉴别,肿瘤扩大切除是治疗KHE的最佳手段。
Purpose To explore the clinicopathologie fea- tures, diagnosis and differential diagnosis of Kaposiform heman- gioendothelioma. Methods To reported the histopathologieal and immunohistochemical features in 29 cases of Kaposiform he- mangioendothelioma, combined with the clinical data and the lit- erature review. Results Of the 29 patients, 15 were male and 14 were female, almost all patients were no more than 1 year old, except one patient aged 25 years. 28 cases occurred in su- perficial skin, including 15 cases in limbs, 5 cases in head and neck, 4 cases in trunk, 1 case in armpit, 3 cases were multiple sites, and 1 case in pelvic cavity. The clinical manifestations were limited or diffuse skin painless plaque, and 16 cases with Kabasaeh-Merritt phenomenon. Microscopically, the tumor was composed of proliferating small blood vessels and spindle cells, with irregular nodular or lobulated distribution, and invasive growth. Spindle cells were arranged in bundles, with epithelioid cells and spindle cells arranged in nests, and glomerulus-like structures, and the latter with crescent shaped vascular fissures. Immunohistochemically, the tumor cells were strongly positive for VEGFR-3, CD31, CD34 and ERG. Partial tumor cells were positive for SMA and D2-40, but negative for CK, Glut-1 and HHVS. Conclusion Kaposiform hemangioendothelioma is a rare, intermediate, locally aggressive angiogenic tumor, mainly occurring in infants and young adults. The diagnosis relies on its unique morphology and immunophenotype, and it should be dif- ferentiated from tufted angioma, infantile hemangioma, congeni- tal hemangioma, spindle cell hemangioma, verrucous venous malformation and Kaposi sarcoma. Extended resection is the best way to treat Kaposiform hemangioendothelioma.
Chinese Journal of Clinical and Experimental Pathology
： Kaposiform hemangioendothelioma
Kabasach-Mer- ritt phenomenon
diagnosis and differential diagnosis