手部Kaposi血管内皮瘤的诊断与治疗

Diagnosis and treatment of Kaposiform hemangioendothelioma of the hand

目的：探讨手部Kaposi血管内皮细胞瘤（ KHE ）的临床特征、诊断与治疗。方法2010年至2013年，我院收治5例手部Kaposi血管内皮瘤患者。男4例，女1例，年龄为6个月至33岁，平均4.6岁（55个月），1例为33岁成年人，余4例均为婴幼儿。本组病例术前检查均未发现凝血系统异常。5例均采取外科手术局部肿物扩大切除。结果术后病理均确诊为Kaposi型血管内皮细胞瘤，肿瘤表现为多结节状侵袭性生长，细胞呈血管内皮细胞样，可见密集分布的梭形肿瘤细胞和筛状或裂隙样血管，局灶增生呈肾小球样结构。部分病例有组织学或影像学证据显示骨破坏。免疫组织化学CD31，CD34，Fli-1蛋白，第VIII因子等血管标记阳性，SMA 灶性阳性，1例局灶性表达 CK。术后随访12～25个月，平均18个月，其中1例术后1年复发，再次接受手术治疗，剩余病例均无复发。结论对于手部单纯KHE的病例，单纯手术扩大切除肿瘤，可获得良好的结果。

Objective To investigate the clinical features and the diagnosis and treatment of Kaposiform hemangioendothelioma （ KHE ） of the hand. Methods From 2010 to 2013, 5 patients with KHE of the hand were treated in our department, including 4 males and 1 female. Their mean age was 4.6 years old （ range;6 months-33 years ）, among whom there was 1 adult patient of 33 years old and 4 infants. No abnormal coagulation function was found before the operation. Extended local resection of tumors were performed on all the patients. Results The diagnosis of KHE was conifrmed by the postoperative pathology. A multiple lobular and invasive growth pattern was observed in the tumors, and vascular endothelial cells were shown. The densely packed spindle-shaped tumor cells and sieve-like or slit-like blood vessels could be seen, with focal hyperplasia of glomeruloid structures. Bone destruction was found in some cases in the histological or radiographic examination. The immunohistochemical staining showed all the vascular markers were positive including cluster of differentiation （ CD ） 31, CD34, （ friend leukemia integration 1 transcription factor ） Fli-1 and Factor VIII. The smooth muscle antibodies （ SMA ） were focally positive and the focal expression of cytokeratin （ CK ） was noticed in 1 case. All the patients were followed up for a mean period of 18 months （ range;12-25 months ）. No recurrence was found but only in 1 patient at 1 year after the operation, who underwent surgery again. Conclusions Simple extended resection of tumors is an effective method in the treatment of KHE of the hand, with excellent outcomes.