Imaging Appearance of Renal Epithelioid Angiomyolipoma: a Rare Variant of Renal Angiomyolipoma with Diverse Clinical Behaviours
PY Chu, WY Wong, WCS Chan, KL Siu
Hong Kong J Radiol 2015;18:287-92
DOI: 10.12809/hkjr1514288
Renal epithelioid angiomyolipoma is a rare variant of renal angiomyolipoma. The condition occurs in patients with or without tuberous sclerosis. Clinical behaviour of renal epithelioid angiomyolipoma varies widely, ranging from benign to malignant. Only a few reports have described its radiological features. We report two cases of renal epithelioid angiomyolipoma in patients without tuberous sclerosis. The first patient had no recurrence after nephrectomy, while the other patient had metastasis at presentation and initially responded to temsirolimus but later showed disease progression.
中文摘要
腎上皮樣血管平滑肌脂肪瘤的影像學表現：腎血管平滑肌脂肪瘤罕見變種呈現廣泛臨床特徵的影像學表現
朱炳容、王慧瑜、陳煥章、蕭廣樂
腎上皮樣血管平滑肌脂肪瘤是腎血管平滑肌脂肪瘤的罕見變種。不論患者是否有結節性硬化症都可以有腎上皮樣血管平滑肌脂肪瘤。此症從良性到惡性的臨床表現可以有很大差異，迄今描述其影像學特徵的文獻較少。本文報告腎上皮樣血管平滑肌脂肪瘤的兩名患者，均無結節性硬化。其中一名患者接受腎切除後無復發。另一名患者病發時出現轉移，初期對西羅莫司有反應，但後來病況加劇。