结节性硬化症患者并发双侧肾血管平滑肌脂肪瘤和肾细胞癌。
Concurrent bilateral renal angiomyolipoma and renal cell carcinoma in a patient with tuberous sclerosis complex.
作者信息
Khallouk Abdelhak, Ahallal Younes, Doublali Mbarek, Tazi Mohamed Fadl, Mellas Soufiane, El Fassi Mohamed Jamal, Farih Moulay Hassan
机构信息
Department of Urology, Hassan II University Hospital Center Fez, Morocco.
出版信息
Rev Urol. 2009 Fall;11(4):216-8.
Abstract
Renal angiomyolipomas (AMLs) are often associated with tuberous sclerosis. These tumors are predominantly benign, although malignant forms do exist and are known to be associated with renal cell carcinoma. This case report describes a patient with tuberous sclerosis and massive bilateral AML. Total right nephrectomy was performed; histopathologic examination revealed the coexistence of AML and clear cell renal carcinoma in the same kidney. Because differentiation between renal cell carcinoma and AML with minimal or no fat component can be difficult, an accurate diagnosis is critical in the management of renal AML.
摘要
肾血管平滑肌脂肪瘤（AML）常与结节性硬化症相关。这些肿瘤大多为良性，不过恶性形式确实存在，且已知与肾细胞癌有关。本病例报告描述了一名患有结节性硬化症和双侧巨大AML的患者。实施了右侧全肾切除术；组织病理学检查显示同一肾脏中存在AML和透明细胞肾细胞癌。由于区分肾细胞癌和脂肪成分极少或无脂肪成分的AML可能存在困难，因此准确诊断对于肾AML的治疗至关重要。
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