CAJ | 학술논문

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.
목적 관찰료해총상혈관류적림상병리특점.방법 대16례총상혈관류환인진행림상병이학관찰,병결합문헌진행토론.결과 16례총상혈관류환인,년령위15 d지11세1개월,평균29.3개월령;남13례,녀3례;기중두부5례,구간3례,지체8례.전부병례균위단발,채용수술절제.림상표현위단발혹다발담홍색혹자홍색적반편、구진、결절혹반괴,가반유심재적피하결절.조직병이학견:진피급피하조직가견성족분포적류양모세혈관총,저배경하정"포탄"양외관,세포무이형성.면역조직화학표기:CD31(+)、CD34(+)、FⅧAg(+)、SMA(+)、VEGF(+)、Vimentin(+)화Glut1(-).수방3개월지7년1개월,부유1례우이후총상혈관류술후3개월복발,개용평양매소류체주사치료,간격1개월,주사3차후전유,지금미견복발.결론 총상혈관류유일정자한성,국부절제가치유,단유복발경향,보수관찰화술후적환인응감측혈소판화섬유단백원수량,약유발현잠재침습성,유향Kaposiform혈관내피류전화경향,응행확대수술절제,술후병리검사학진.
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.