侵袭性血管黏液瘤临床病理分析

目的 总结侵袭性血管黏液瘤（AAM）的临床表现、病理特点、诊断和治疗.方法 利用PubMed和CHKD数据库检索系统,以“侵袭性血管黏（或粘）液瘤”为关键词,检索所有有关文献.选择符合检索条件的210篇参考文献,分别对AAM有关的病史、临床表现、诊断、病理特点、治疗及预后的文献进行分析总结.结果 282例AAM中男64例,女218例,男女之比为1：3.4.年龄1～83岁,平均40.38岁.AAM好发于生育期女性外阴、生殖道及盆腔软组织,男性阴囊、精索、腹股沟等处.术前不能明确诊断.肿瘤直径最小1 cm,最大60 cm.所有病例都有典型的病理特征.免疫组织化学表明Vimentin、CD34、Desmin、雌激素受体、孕激素受体表现强阳性,而Ki-67、S-100、Actin则大多数为阴性.病程1个月至20年.术后复发时间2个月至20年.本病确诊需依靠病理学检查.结论 AAM是一种罕见的软组织肿瘤,原因不明,病程进展缓慢,具有局部侵袭性,切除后易复发,需要长期随访.

Clinical and pathological analysis of aggressive angiomyxoma

Objective To summarize the clinical manifestations,pathological character,diagnosis and treatment of aggressive angiomyxoma （AAM）.Methods A computer-based online search of PubMed database and CHKD database was undertaken for literature about AAM published from all the relevant documents with the key words of ＂aggressive angiomyxon＂.According to the condition 210 articles were analyzed.All the articles were analyzed about natural history,clinical manifestation,diagnosis,pathological character,treatment and prognosis of AAM.Results A total of 282 cases in well-documented articles had been reported,among which 64 were male and 218 were female,with male to female ratio of 1：3.4.The age of the patients from 1 to 83 years（mean 40.38 years）.The most common sites were the perineum,genital tract and soft tissue in pelvic cavity in females and the scrotum,spermatic cord and groin in males.None of the cases could be accurately diagnosed as AAM preoperatively.The minimum diameter of the tumors was 1 cm,and the maximum was 60 cm.All the specimens showed typical pathological features of AAM as reported previously.Immunohistochemistry indicated that AAM tended to be strongly positive for vimentin,CD34,Desmin,estrogen receptor,progesterone receptor but mostly negative for S-100 and Ki-67 and Actin.The medical history was from 1 month to 20 years.The recurrence of the postoperative follow-up was 2 months to 20 years.The diagnosis depended on pathological examination.Conclusions AAM is a sort of unusual soft connective tissue tumor.It is a kind of unknown cause,slow progression,locally invasive,easy to recur after tumor resection.Long-term follow-up is quite necessary because of the high rate of local recurrence.