丛状血管瘤的临床病理学观察

Clinico-pathologic study of tufted angioma

目的 观察了解丛状血管瘤的临床病理特点.方法 对16例丛状血管瘤患儿进行临床病理学观察,并结合文献进行讨论.结果 16例丛状血管瘤患儿,年龄为15 d至11岁1个月,平均29.3个月龄;男13例,女3例;其中头部5例,躯干3例,肢体8例.全部病例均为单发,采用手术切除.临床表现为单发或多发淡红色或紫红色的斑片、丘疹、结节或斑块,可伴有深在的皮下结节.组织病理学见：真皮及皮下组织可见成簇分布的瘤样毛细血管丛,低倍镜下呈＂炮弹＂样外观,细胞无异型性.免疫组织化学标记：CD31（＋）、CD34（＋）、FⅧAg（＋）、SMA（＋）、VEGF（＋）、Vimentin（＋）和Glut1（-）.随访3个月至7年1个月,仅有1例右耳后丛状血管瘤术后3个月复发,改用平阳霉素瘤体注射治疗,间隔1个月,注射3次后痊愈,至今未见复发.结论 丛状血管瘤有一定自限性,局部切除可治愈,但有复发倾向,保守观察和术后的患儿应监测血小板和纤维蛋白原数量,若有发现潜在侵袭性,有向Kaposiform血管内皮瘤转化倾向,应行扩大手术切除,术后病理检查确诊.

Objective To evaluate the clinic-pathologic characteristics of tufted angioma. Methods The clinicopathologic data were reviewed in 16 cases of tufted angioma in children. Results Sixteen children with tufted angioma,aged 15 days -11 years and 1month （mean age of 29. 3 months） were reviewed. Of these,13 were male and 3 were female. Five angioma were located in the head,3 in the trunk,and 8 in limbs. All tumors were single and were surgically removed. Clinical presentations include single or multiple plaques light red or purple in color, papules,or nodules which may be associated with subcutaneous nodules. Histopathology revealed a tumor-like distribution of visible clustered capillary plexus in dermis and subcutaneous tissue. Low power microscopy showed a ＂cannon-ball like＂appearance. The cells have no atypia. Immunohistochemical staining showed that the tumors were CD31（ ＋ ）,CD34 （ ＋ ）,SMA （ ＋ ）,VEGF （ ＋ ）, Vimentin （ ＋ ） and Glutl （ - ）. Patients were followed-up for 3 months to 7 years and l month. One case recurred 3 months after surgery. Utilizing the intra-lesion injection of Pingyangmycin at one month intervals, the patient recovered after three injections. Up to date, there has been no further recurrence. Conclusions The surgery is feasible for tufted angioma though there is a tendency of recurrence. Post-operatively, platelets and fibrinogen should be monitored.