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目的探讨侵袭性血管黏液瘤(aggressive angiomyxoma,AAM)的临床病理学特征、鉴别诊断及治疗方法。方法对3例AAM的临床病理特点进行回顾性研究,采用免疫组织化学染色,检测AAM组织中雌激素受体(ER)、孕激素受体(PR)、结蛋白(desmin)、平滑肌肌动蛋白(SMA)、波形蛋白(VIM)、S-100、CD34和CD117的表达水平,并对其治疗及预后进行随访。结果 3例AAM患者均为女性,年龄27~44岁,肿瘤发生于外阴或盆腔,3例均表达desmin、VIM、ER、PR,SMA在2例呈阳性表达,1例为阴性表达。所有病例均不表达S-100、CD34及CD117。3例患者均行肿瘤局部切除术,随访43~70月,其中1例患者于随访30月时复发一次,所有患者随访至今均无瘤存活。结论 AAM是一种少见肿瘤,容易局部复发,但不发生转移,组织学表现及免疫组织化学染色对其病理诊断具有重要作用,手术切除是目前主要的治疗方式,患者应长期随访。
Objective To investigate the clinicopathological features, differential diagnosis and treatment of aggressive angiomyxoma (AAM). Methods The clinical and pathological features of 3 cases of AAM were retrospectively studied. The expression of estrogen receptor (ER), progesterone receptor (PR), desmin, smooth muscle actin (SMA), vimentin (VIM), S-100, CD34 and CD117 were detected and their treatment and prognosis were followed up. Results All 3 AAM patients were female, aged from 27 to 44 years old. The tumors occurred in the vulva or pelvis. The expression of desmin, VIM, ER, PR and SMA were all found in 3 cases and negative in 1 case. All cases did not express S-100, CD34 and CD117.3 patients underwent partial tumor resection, followed up for 43 to 70 months, of which 1 case of recurrence at follow-up 30 months, all patients were followed up so far tumor-free survival. Conclusions AAM is a rare tumor which is easy to local recurrence, but does not metastasize. Histological examination and immunohistochemical staining play an important role in the pathological diagnosis. Surgical resection is the main treatment and patients should be followed up for a long time.