肾上皮样血管平滑肌脂肪瘤1例报告及文献复习

Renal epithelioid angiomyolipoma:a case report and literature review

目的:探讨肾上皮样血管平滑肌脂肪瘤(EAML)的临床病理特征、诊断、鉴别诊断、治疗方法及预后。方法:通过观察1例肾EAML患者的临床病理学表现与免疫组化标记并复习有关文献,讨论其组织学特征及临床生物学行为。结果:CT平扫呈略高密度实性肿块,边界清楚,病灶内无明显脂肪密度;增强扫描密度相对均匀,实质期强化程度最高,排泄期密度下降较少。镜检示肿瘤细胞弥漫分布,瘤体主要成分为上皮样细胞,细胞异型性明显,大小形状不等,核大且染色明显加深,偶有多核巨细胞,核分裂像明显。免疫组化染色显示瘤细胞HMB45+,SMA+,CK-。结论:病史、体检结合影像学检查能够给我们提供重要的诊断线索,组织病理学特点结合免疫组化标记有助于该疾病的确诊,治疗方法以手术切除为主,对于预后较差的患者应按照肾细胞癌标准长期随访。

Objective： To investigate the clinicopathological features, diagnosis, differential diagnosis, treat-ment and prognosis of renal epithelioid angiomyolipoma （EAML）. Method： A case of renal EAML was analyzed by clinicopathological examination and immunohistochemistry. The related literature was reviewed and the histo-logic features of renal EAML and the clinical biologic behavior were discussed. Result： Unenhanced CT showed that slightly hyperdense lump with clear boundary and no fat could be detected. The mass was enhanced obviously in the parenchymal phase and slightly decreased in excretory phase. Pathological examination showed that tumor was mainly composed of epithelioid cells with invasive hyperplasia of atypical pleomorphism, hyperchromatic nuclei with frequent mitosis, multinucleated giant cells. Immunohistochemically, the neoplastic cells were positive for HMB45 and SMA, but negative for CK. Conclusion： Medical history and physical examination combined with i-conographic data can provide us important clues to the diagnosis. Pathological characteristics combined with immu- nohistochemistry are critical in the final diagnosis. Surgical resection should be the first choice. Long-term follow-up plan should be carried out for some patients with poor prognosis according to the renal cell carcinoma standard guidelines.