Aubert J, Doré B, Orget J
J Urol (Paris). 1985;91(9):575-80.
Four patients presented isolated angiomyolipomas of kidney in the absence of any form of phakomatosis such as Bourneville's disease. The tumor was removed by partial nephrectomy in only one case, after histology of a fresh specimen confirming the presence of an angiomyolipoma without signs of cancer. In another patient it was impossible to perform conservative surgery because of dissemination of the angiomyolipoma throughout the kidney. Symptomatology suggestive of the disorder includes lumbar pain, kidney tumor and more rarely hematuria. Diagnosis is difficult despite availability of ultrasound and CT scan imaging, which has supplanted renal arteriography. Confirmation requires histology of a fresh specimen. Operative treatment is essential to avoid the risk of intra- and peri-tumoral hemorrhage, particularly during pregnancy. Partial nephrectomy or excision of tumor is possible when the diagnosis has been established, if excision involves healthy parenchyma and if after strict hemostasis at least one third of renal parenchyma remains.
4例患者出现孤立性肾血管平滑肌脂肪瘤，且不存在任何形式的错构瘤病，如结节性硬化症。仅1例患者在新鲜标本组织学检查证实为无癌症迹象的血管平滑肌脂肪瘤后，通过部分肾切除术切除了肿瘤。在另1例患者中，由于血管平滑肌脂肪瘤弥漫于整个肾脏，无法进行保守手术。提示该疾病的症状包括腰痛、肾肿瘤，较少见的是血尿。尽管有超声和CT扫描成像（已取代肾动脉造影），但诊断仍很困难。确诊需要新鲜标本的组织学检查。手术治疗对于避免肿瘤内和肿瘤周围出血的风险至关重要，尤其是在妊娠期。如果诊断已明确，且切除涉及健康实质，并且在严格止血后至少保留三分之一的肾实质，则可行部分肾切除术或肿瘤切除术。
