2013-11046-颅底Kaposi型血管内皮瘤1例并文献复习4.doc

颅底Kaposi型血管内皮瘤1例并文献复习 汪明云1 魏荣富1 周青2 (1.南京高淳区人民医院肿瘤内科211300；2.江苏省肿瘤医院210009) 【摘要】目的：原发于颅底的Kaposi型血管内皮瘤(KHE)罕见，本文报道1例，探讨颅内KHE的诊治。方法：对1例确诊为颅内Kaposi型血管内皮瘤的诊治，复习相关文献，就本病临床表现、病理特征、诊断、治疗及预后进行分析讨论。结果：颅底Kaposi型血管内皮瘤属间叶性肿瘤，生物学行为属交界性或不确定性肿瘤。肿瘤由排列呈巢状、索状的上皮样细胞组成，内含血管腔，核分裂象少见。免疫组织化学检测显示CD31、CD34、FⅧ阳性。手术切除是其主要治疗方法，放疗敏感，预后较好。结论：颅底Kaposi型血管内皮瘤罕见，其生物学行为具有潜在侵袭性，建议尽可能完全切除，术后宜行瘤床区局部放疗，根据病情可选择化疗联合抗血管生成治疗，继之抗血管生成维持治疗。 【关键词】颅底肿瘤；血管内皮瘤；Kaposi型血管内皮瘤. Intracranial Kaposiform Hemangioendothelioma：Case Report and Literature Review WANG Mingyun,WEI Rongfu,ZHOU Qing (1.Department of oncology medicine，GAOCHUNPEOPLE’SHOSPITAL，211300，China；2.Department of oncology medicine JIANGSU PROVINCE CANCER HOSPITAL，210009，China) 【Abstract】 Purpose:Primary intracranial Kaposiform haemangioendotheioma(KHE) is rare.To study the treatment of intracranial KHE.Methods:By case study and literature review，clinical and pathological features，diagnosis，treatment and prognosis of intracranial KHE.Results:Based of our report and review of the literature,KHE is rare and with border lineoruncertain behavior.The tumor usually appeared as a small nest or cords composed of eosinophilicepi thlioidorspindled cells.Containing lumen and lack of pleomorphism.Inmmnohistochemical stain showed that the tumour cells were positive for CD34,CD31,FⅧ.KHE may be treated with complete surgical resection whenever possible and is sensitive to radiation.The KHE has a favorable prognosis.Conclusions：KHE has variable malignant potential.Surgeryis the first choice therapy.postoperative radiotherapy should be usedfor the tumor beda rea.Chemical therapy combined anti angiogenesis therapy and maintenance treatment of anti angiogenes therapy. 【Keywords】intracranial；haemangioendothe1ioma；Kaposiform 卡波西形血管内皮瘤(Kaposiform Hemangioendothelioma，KHE)是一种罕见的组织形态类似于卡波西肉瘤且具有侵袭性的肿瘤，好发于儿童和婴幼儿，男女发病率无差异，1993年由Zukerberg等首先描述。2002年WHO软组织和骨肿瘤分类将其划分为交界性或生物行为未定的肿瘤。KHE多发生于四肢的皮肤及深部软组织【1】，由不成熟血管构成，主要含有卡波西肉瘤样结节状、束状梭形细胞结构。肿瘤周边组织常受侵及，无自发消退倾向，呈侵袭性生长，仅有局部淋巴结转移，尚未见远处转移的报道。常伴有卡梅综合