6例血管平滑肌脂肪瘤临床病理观察

摘要

目的 探讨血管平滑肌脂肪瘤(Aml)的临床病理学特点.方法 通过6例Aml[其中1例上皮样血管平滑肌脂肪瘤(EAml)]临床病理观察与免疫组化标记,探讨其组织学特征及临床生物学行为.结果 5例年龄分别为52、51、44、46、59岁,肿瘤发生在右肾(其中1例为双侧,左侧因肿瘤巨大并大出血,先左肾动脉介入治疗止血,后做右肾肿物切除);另1例50岁,肿瘤位于子宫旁.5例为经典型Aml,1例EAml.EAml患者因大出血休克,经抢救无效死亡.结论 Aml为良性间叶性肿瘤,EAml认为是具有恶性潜能肿瘤,两者均有不同比例的血管平滑肌脂肪组织成分,其中常见血管纤维素样变性,血管破裂合并出血,严重者危及患者生命,应提醒临床医生及早干预.%Objective To investigate the clinicopathological features of angiomyolipoma (Aml).Methods Six cases of Aml (one of them EAml) were observed clinicopathologically and immunohistochemically,investigating their histological characteristics and clinical biological behaviors.Results The ages of 5 of the 6 cases were 52,51,44,46,and 59;they had tumor on the right kidney (one of them was bilateral,because of huge tumor and massive hemorrhage on the left side,interventional therapy of left renal artery was performed to stop bleeding,and then right renal mass resection was performed).The other patient was 50 years old and her angiomyolipom was located near the uterus.There were 5 cases of classical type and 1 epitheloid type.The patient diagnosed as EAml died of massive hemorrhagic shock.Conclusions Aml is a benign mesenchymal neoplasm.EAml is a malignant potential neoplasm.Both of them have different proportion of vascular smooth muscle adipose tissue components.Among them,vascular cellulose degeneration,vascular rupture,and bleeding are common,and seriously endanger the patients' lives.Clinicians should be reminded to intervene early.