201311046-颅底K拟aposi型血管内皮瘤1例并文献复习4.docVIP

颅底Kaposi型血管内皮瘤Kaposi型血管内皮瘤KHE的诊治。方法：对1例确诊为颅内Kaposi型血管内皮瘤Kaposi型血管内皮瘤Kaposi型血管内皮瘤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。常伴有卡梅综合征(Kasabach-Merritt syndrome，KMS) (巨大毛细血管瘤、血小板减少性紫癜、纤维蛋白原缺乏血症)。由于KHE发病率低，与多个从良性到恶性的肿瘤或病变有交，易误诊误治。出血、感染或影响重要结构是死亡的常见原因。发生于颅内的卡波西样血管内皮细胞瘤经文献检索未见报道。作者遇到1例，主要对诊疗及预后判断做进一步探讨。 1.临床资料 患女9岁2009年出现头痛，外院查头颅MR未见异常，后头痛反复发作，缓慢加重，至就诊查头颅MR示前颅底、斜坡占位病变，病变累及鞍底、蝶窦、海绵窦，边界不清，2011-06-30南京军区总院行鼻蝶窦、斜坡肿瘤切除术，术后病理病理经多院会诊考虑：（前颅底）中间型或低度恶性的血管内皮瘤。2011-08-01残余肿瘤调强放疗，D