肾上皮样血管平滑肌脂肪瘤一例报告并文献复习

目的 探讨肾上皮样血管平滑肌脂肪瘤(EAML)的病理及影像学特点.方法 患者,男,47岁.左上中腹疼痛不适1年入院.查体左上腹部可扪及巨大质韧肿块,叩痛(+).CT检查左肾见一最大径约28 cm肿块伴腹主动脉旁多枚肿大淋巴结,增强后呈不均性中等度强化.平扫相对高密度;乏脂肪;强化方式呈"快进慢出".全麻下行根治性左肾切除及周围淋巴组织清扫术.检索Ovid和CBM数据库相关文献复习讨论. 结果病理检查:肿瘤包膜完整,来源于左肾中上部,不能与左肾完全分离;切面为鱼肉样组织,伴灶性坏死组织;镜下:密集分布的上皮样细胞,无脂肪组织;免疫组化染色黑色素瘤相关抗原(HMB-45)强阳性、上皮膜抗原(EMA)阴性.病理报告:左肾EAML伴腹腔淋巴结转移,输尿管残端、血管残端及肾周组织未见肿瘤浸润.术后随访1年肿瘤未见复发转移.结论 EAML是一种罕见的、有恶性潜能的问叶肿瘤,有一定的病理及影像学特点,预后相对较好.

A case report of epithelioid angiomyolipoma and review of the literature

Objective To explore the pathological and imaging features of epithelioid angiomyolipoma(EAML). Methods A 47-year-old man presented with dull pain in left upper abdomen for 1 year admitted to hospital.Accompanied with several enlarged lymph nodes,a mass with the largest diameter of 28 cm in the left kidney was inhomogeneously enhanced on CT.The left kidney and the mass were radically resected with regional lymph node dissection after general anaesthesia. Results During the operation,a well-circumscribed,encapsulated mass with several paraaortic lymph nodes was found on the upper-middle left kidney.The gross cut surface showed fleshy texture with regions of necrosis.Microscopically and immunohistochemistrically,diffuse sheets of epithelioid cells were found with positivity for HMB-45 and negativity for EMA.CT features included higher pre-contrasted density,absent fat,enhanced mode of quick wash-in and slow wash-out.There was neither metastasis nor recurrence during the 1 year follow-up. Conclusion EAML with malignant potency is a rare tumor of mesenchymal tissue presenting with some specific radiopathological features and a good prognosis.