肾上腺血管平滑肌脂肪瘤临床特征分析

Clinical characteristics of rare adrenal angiomyolipoma

目的探讨肾上腺血管平滑肌脂肪瘤的临床特征。方法回顾性分析四川大学华西医院2009年4月至2019年4月收治的5例肾上腺血管平滑肌脂肪瘤患者的临床资料。男2例,女3例;年龄平均46.7(40~57)岁。1例临床表现为右上腹包块,1例腰背部胀痛,余无显著症状;1例合并高血压,1例合并肾上腺素、去甲肾上腺素、肾素活性(立位)降低,其余病例激素未见异常。2例行腹部B超检查示肾上腺区强回声团;5例均行腹部增强CT检查,示肾上腺区占位性病变,具体表现为脂肪、血管、肌肉等混杂密度的肿瘤。肿瘤直径平均5.8(2.3~9.1)cm,其中左侧2例,右侧3例;合并肾血管平滑肌脂肪瘤1例。5例均行腹腔镜肾上腺肿瘤切除术。总结患者术后情况及预后。结果5例手术均顺利完成,术后病理均诊断为肾上腺血管平滑肌脂肪瘤。合并高血压患者术后血压水平无明显变化。随访时间2~7年,5例肿瘤均无复发。结论肾上腺血管平滑肌脂肪瘤是一种极其罕见的良性疾病,多于中年发病,患者多无明显临床症状及体征;肿瘤通常不分泌有临床意义的激素,结合CT等影像学检查可基本诊断,确诊依赖于病理;术后预后良好。

Objective To analyze the clinical characteristics of rare adrenal angiomyolipoma.Methods Clinical data of 5 patients with adrenal angiomyolipoma admilted to West China Hospital of Sichuan University from April 2009 to April 2019 were retrospectively analyzed.There were 2 males and 3 females.Age ranged from 40 to 57 years old,with an average of 46.7(40-57)years old.Clinical manifestations included right upper abdominal mass in 1 case,pain in the lower hack in 1 case,and no significant symptoms were found in the rest.One patient was complicated with hypertension,one patient was complicated with decreased activities of epinephrine,norepinephrine and renin activity(orthosis),and the other patients had no abnormal hormones.2 patients underwent abdominal ultrasound with"strong echo mass in adrenal area",and all underwent enhanced abdominal CT with"spare occupying lesion in adrenal area",which was specifically manifested as tumors with mixed density in fat,blood vessels,muscle and so on.The average tumor diameter was 5.8(2.3-9.1)cm,including 2 cases on the left,3 cases on the right,and 1 case with renal angiomyolipoma.All patients underwent laparoscopic adrenal tumor resection.Results All the 5 patients undement surgical resection successfully and were diagnosed as adrenal angiomyolipoma hy pathological examination of tumor specimens after surgery.There was no significant change in blood pressure level of patients with hypertension after surgery.Follow-up time was 2-7 years and no recurrence.Conclusions Adrenal angiomyolipoma is an extremely rare benign nonfunctional disease,which is more common in middle age.Most of the patients had no clinical histon and signs.The tumor has no hormone secretion function and can he basically diagnosed with CT and other imaging examinations.The final diagnosis depends on pathological examination.After operation,the prognosis of adrenal angiomyolipoma is good.