Epithelioid Angiomyolipoma of the Kidney Mimicking Renal Cell Carcinoma: A Clinicopathologic Analysis of Cases and Literature Review

與腎細胞癌相似之類上皮腎臟血管肌肉脂肪瘤－病例分析回顧

蔡嘉駿(Chia-Chun Tsai) ； 吳文正(Wen-Jeng Wu) ； 李經家(Ching-Chia Li) ； 王起杰(Chii-Jye Wang) ； 黃俊雄(Chun-Hsiung Huang) ； 吳俊杰(Chun-Chieh Wu)

《The Kaohsiung Journal of Medical Sciences》 25卷3期 (2009/03) Pp. 133-140

摘要

典型腎臟血管肌肉脂肪瘤為常見腫瘤，大多呈現良性病灶且有典型影像及病理學特徵。但少數類上皮腎臟血管肌肉脂肪瘤於臨床有惡性表現。我們回顧自2000年以來在高雄醫學大學附設中和紀念醫院中所診斷的五位類上皮腎臟血管肌肉脂肪瘤病例。所有病人經電腦斷層掃描均被診斷為腎細胞癌且沒有其他系統性的轉移。組織切片的結果顯示腫瘤細胞與高度未分化惡性肉瘤腎細胞癌相似。最終診斷依靠發現類上皮細胞的存在及免疫組織化學的標示。手術後在持續追蹤之下，並沒有疾病復發。類上皮腎臟血管肌肉脂肪瘤可能於臨床有惡性表現。此種腫瘤須靠組織切片的成像及免疫組織化學的標示來與惡性腎細胞癌作鑑別診斷。經統計近三分之一的類上皮腎臟血管肌肉脂肪瘤有有惡性表現，故對於此種腫瘤所採取的治療及追蹤方式應和腎細胞癌相似。

關鍵字

類上皮變化 ； 腎臟血管肌肉脂肪瘤

並列摘要

Classical renal angiomyolipoma (AML) is a common tumor that, in most cases, follows a benign course and has clearly defined radiologic and histological characteristics. However, rare cases of clinically aggressive or malignant AML, the epithelioid variant of AML (EAML), have been reported. Here, we review the five cases of EAML diagnosed since 2000 at our institution to highlight the spectrum of clinical, radiological and histological characteristics. In all cases, renal lesions seen on computed tomography (CT) were considered as suspicious for renal cell carcinoma (RCC) without tumor extension or metastasis. One of the two patients with definitive tuberous sclerosis complex had a small conventional AML with fat content in the other kidney. Histologically, these tumors can resemble sarcomatoid RCC or high grade RCC. The final diagnosis is established by the presence of perivascular epithelioid cells and immunohistochemistry markers. There was no evidence of local recurrence or metastatic disease found by sonography, CT and magnetic resonance imaging during the follow-up period. EAMLs are capable of aggressive or malignant clinical behavior. Approximately one third of the reported EAMLs show advanced disease; therefore, we recommend that it is important to treat and closely follow-up such cases, similar to that for RCC, because of its malignant potential.

並列關鍵字

epithelioid variant ； renal angiomyolipoma