Tong Y C, Chieng P U, Tsai T C, Lin S N
Department of Urology, Medical College, National Cheng Kung University, Tainan, Taiwan, Republic of China.
Br J Urol. 1990 Dec;66(6):585-9. doi: 10.1111/j.1464-410x.1990.tb07187.x.
A series of 24 patients with renal angiomyolipoma was reviewed. Their ages ranged from 10 to 70 years (average 41). Four cases were associated with tuberous sclerosis, 1 with spinal neurilemmoma, 1 with transitional cell carcinoma and another with renal tuberculosis. The presenting symptoms in decreasing frequencies were flank pain, mass, haematuria, fever, syncope and respiratory distress. Although it has often been said that angiomyolipomas associated with tuberous sclerosis are small and asymptomatic, all 4 such patients in this study had large symptomatic tumours. Before the advent of CT scan and ultrasonography, the pre-operative diagnostic rate for cases unassociated with tuberous sclerosis was 10% (1/10). With the combined use of these 2 modalities, the diagnostic rate increased to 60% (6/10). Most patients in this series were treated with nephrectomy. However, equally good outcomes occurred in 2 patients who received partial nephrectomies. Pre-operative diagnosis now makes it possible to consider more conservative management.
回顾了24例肾血管平滑肌脂肪瘤患者。他们的年龄在10岁至70岁之间（平均41岁）。4例与结节性硬化症相关，1例与脊髓神经鞘瘤相关，1例与移行细胞癌相关，另1例与肾结核相关。出现频率由高到低的症状依次为胁腹痛、肿块、血尿、发热、晕厥和呼吸窘迫。尽管常说与结节性硬化症相关的血管平滑肌脂肪瘤体积小且无症状，但本研究中的所有4例此类患者均有较大的有症状肿瘤。在CT扫描和超声检查出现之前，与结节性硬化症无关的病例术前诊断率为10%（1/10）。联合使用这两种检查方法后，诊断率提高到了60%（6/10）。本系列中的大多数患者接受了肾切除术。然而，2例行部分肾切除术的患者也取得了同样良好的效果。现在术前诊断使得考虑更保守的治疗成为可能。
