Gattegno B, Catala M, Tenaillon M, Mignard J P, Coloby P, Meyrier A, Thibault P
J Urol (Paris). 1981;87(2):87-91.
The authors report a quite exceptional case of isolated angiomyolipoma of one kidney in the absence of any manifestation of phakomatosis. Discovery of the tumour led to special investigations which confirmed the solid and hypervascularised nature of the tumour. A doubt remained with regard to its precise nature, and hence an extended nephrectomy, though without damage to the vessels at the time of section. The kidney was cooled and preserved by perfusion which allowed time for adequate histological study. The diagnosis of angiomyolipoma being confirmed, without the slightest risk of dissemination if by chance it had not been a benign tumour, the kidney was transplanted into the lumbar fossa via its own vessels with an excellent result. The authors discuss two problems on the basis of this case: 1) That of preoperative diagnostic difficulties: the scanner provides valuable information when it reveals the presence of intra-tumoral fatty tissue. 2) Difficulties in management when the benign nature of the lesion cannot be confirmed before histological examination. The solution adopted: routine extended nephrectomy with ex-vivo perfusion of the kidney making it possible to obey the basic rules of oncology. Frozen section biopsy confirms the diagnosis of angiomyolipoma. It is then possible to auto-transplant the kidney after tumorectomy.
作者报告了一例非常特殊的病例，即一侧肾脏孤立性血管平滑肌脂肪瘤，无任何错构瘤病表现。肿瘤的发现促使进行了特殊检查，证实了肿瘤的实性及血运丰富的特性。但其确切性质仍存疑问，因此实施了扩大肾切除术，术中切面时未损伤血管。通过灌注对肾脏进行冷却和保存，以便有时间进行充分的组织学研究。血管平滑肌脂肪瘤的诊断得到证实，即便万一它不是良性肿瘤也不存在丝毫播散风险，于是将肾脏通过其自身血管移植至腰窝，效果极佳。作者基于此病例讨论了两个问题：1）术前诊断困难：当扫描显示肿瘤内存在脂肪组织时，它能提供有价值的信息。2）在组织学检查前无法确认病变良性性质时的处理困难。所采取的解决办法：常规扩大肾切除术并对肾脏进行体外灌注，从而能够遵循肿瘤学的基本规则。冰冻切片活检证实为血管平滑肌脂肪瘤。然后在肿瘤切除术后可行肾脏自体移植。
