肾上皮样血管平滑肌脂肪瘤的临床特征分析

Clinical features of renal epithelioid angiomyolipoma

目的探讨肾上皮样血管平滑肌脂肪瘤（epithelioedangiomyolipoma，EAML）的病理特点和临床特征。方法回顾性总结2001年2月至2010年7月4例。肾EAML患者的诊治情况和病理特点，结合免疫组化染色检查结果对其预后进行探讨。4例患者中，男2例，女2例。年龄分别为21、62、34、34岁。例1、例2以肿瘤破裂出血就诊，其中例1同时合并多发性结节硬化（tuberoussoleFosiscomplex，TSC），例2出现失血性休克；例3同时合并对侧肾脏透明细胞癌；例4以双肾巨大血管平滑肌脂肪瘤就诊。4例CT检查均发现肿瘤内脂肪成分。结果例1行右肾区肿物探查术，术后6个月失访；例2急诊行右肾切除术，随访10个月未见肿瘤复发；例3首先行右肾癌肿瘤剜除术，6个月后行左肾肿瘤剜除术，随访8个月未见肿瘤复发；例4行双侧减瘤手术，术后6年出现左肾肿痛恶性变，行左肾根治性切除术，随访1年发现全身多发转移，现仍在随访中。4例肿瘤病理诊断均为EAML，免疫组化染色检查示肿瘤细胞HMB45和Melan—A弥漫阳性，而上皮细胞标记不同分子质茸的CK和EMA均为阴性。结论肾EAML是一种少见的具有恶性潜能的肿瘤，对此类患者应根据具体情况采用相应的手术方式，术后密切随诊。病理及免疫组化染色检查对本病的诊断、鉴别诊断以及预后判断有重要的作用。

Objective To explore the pathological and clinical features of renal epithelioid angiomyolipoma （ EAML）. Methods From February 2001 to June 2010, 4 cases of renal epithelioed angiomyolipoma （EAML） diagnosed in our unit were reviewed on their clinical and histopathological feathers retrospectively. The prognosis of renal EAML were evaluated according to the immunohistoehemical findings. Of the 4 cases, the first case had a history of nodular sclerosis and presented a sign of hemorrage caused by the rup- ture of the tumor. The second case also showed a sign of hemorrage complicated with hemorrhagic shock. The third case had no symptom, but suffered from clear cell carinoma concomitantly on the other kidney. The fourth case came for consultation for his bilateral angiomyolipoma. All the 4 cases had fat composition founded in their CT scans. Results The first case was taken biopsy on the tumor of her fight kidney, and lost of follow-up 6 months later. The second case undertaken radical nephrectomy, and no tumor recurrence detected after 10 months follow-up. The third case undertaken fight nephron-sparing nephrectomy, and left nephron-sparing nephrectomy 6 months later. No recurrence detected during the follow-up 8 months later. Bilateral resection of the angiomyolipoma was performed on the fourth case, and a malignancy on the left kid- ney was deteceded 6 years later, then a radical nephrectomy was performed. But liver metastasis was foun- ded half a year later during the follow-up. Immunohistochemcial staining of these tumors showed positive HMB45 and Melan-A but negative CK and EMA. Conclusions Renal EMAL has malignant potentiality, and the aggressiveness of it makes the close follow-up compulsive. Immunohistochemeial study is useful for diagnosis and differential diagnosis, even helpful to assess the prognosis.