摘要目的 探讨经典型和上皮样型肾血管平滑肌脂肪瘤各临床病理特征之间的差异,明确各临床病理特征与生物学行为之间的关系,为临床后续治疗提供理论指导.方法 复查解放军总医院2004至2011年间诊治的肾血管平滑肌脂肪瘤的临床及病理资料,结合随访资料进行统计学分析.结果 8年间共诊治肾血管平滑肌脂肪瘤414例,占同期肾实质肿瘤的8.1%(414/5 287).414例中男性122例,女性292例,男女比例为1.0∶2.4;平均年龄44.0岁(15~74岁).195例发生于左肾(47.1%),右肾212例(51.2%),7例(1.7%)双肾同时发现肿瘤.临床上大多因季肋部疼痛、血尿、可触及包块等而被发现.大体检查见肿瘤大小为0.3~31.0 cm不等,平均6.2 cm;组织学上394例(95.2%)表现为经典型,20例(4.8%)为上皮样型;伴出血、坏死及囊性变者分别为54、23及7例;5、30、14例分别出现肾周脂肪侵犯、核异型细胞及多形核/巨核肿瘤细胞.统计学分析表明核异型细胞的出现概率与肿瘤类型有关,上皮样型更常见到;其余临床病理参数与组织学类型无关.360例获得随访资料,随访时间为3~99个月,1例死于肾细胞癌,其余病例均基本情况良好,无复发及转移.结论 血管平滑肌脂肪瘤是肾实质常见肿瘤之一,临床上基本呈良性生物学经过.组织学上主要表现为经典型和上皮样型;病理诊断时上皮样型者需要与经典型肾细胞癌、MitF家族基因易位相关性肾肿瘤以及肾血管母细胞瘤相鉴别;两种类型肿瘤相比,上皮样型中更常见到核异型细胞,但不影响患者预后.

abstractsObjective To study the different clinicopathological characteristics between classic and epithelioid renal angiomyolipoma, and the relationships between clinicopathological characteristics and biological behaviors as basis for clinical treatment.Methods The clinicopathological and follow-up data for the patients diagnosed with renal angiomyolipoma between 2004 and 2011 were retrospectively reviewed and analyzed.Results There were 414 cases of renal angiomyolipoma diagnosed over 8 years ago, accounting for 8.1% (414/5 287) of all renal parenchymal tumors.The patients included 122 male and 292 female (male-to-female ratio of 1.0∶2.4), mean age 44.0 years (range 15-74 years).Of these, 195(47.1%) tumors occurred in the left kidney, 212(51.2%) in the right kidney and seven (1.7%) were bilateral.Clinically, some cases presented with hypochondrial pain, hematuria or palpable masses.Histologically, 394(95.2%) were classic angiomyolipoma, 20(4.8%) were epithelioid angiomyolipoma;54, 23 and 7 cases had hemorrhage, necrosis and cystic degeneration, respectively;and 5, 30 and 14 cases had perirenal fat invasion, atypical cells and polymorphic/giant tumor cell.There was a positive correlation between atypical cells and epithelioid tumor type, respectively.The other clinicopathological parameters did not correlate with histological type.Follow-up data was available in 360 patients, with follow-up period of 3 to 99 months.One case died from other causes.The remaining patients were free of disease.ConclusionsAngiomyolipoma is a common renal parenchyma tumor.Clinically, it is usually biologically benign.Histologically, it can be either classic or epithelioid types.The epithelioid type should be differentiated from the classic renal cell carcinoma, Mit family translocation renal tumor and renal hemangioblastoma.Atypical cells, more commonly found in the epithelioid angiomyolipoma, do not affect the clinical prognosis of patients.