Renal Angiomyolipoma in association with pulmonary lymphangioleiomyomatosis
Renal Angiomyolipoma in association with pulmonary lymphangioleiomyomatosis
复制标题
DOI：
10.1016/s0272-6386(03)00006-4
复制
发表时间：
2003-04-01
影响因子：
13.2
通讯作者：
De Vriese, AS
中科院分区：
文献类型：
--
作者：
De Pauw, RA;Boelaert, JR;De Vriese, AS
作者报告了一位患有复发性气胸的绝经前女性血液透析患者，该患者被诊断为肺淋巴管平滑肌瘤病 (LAM)。十年前，她因肾肿瘤（相当于血管平滑肌脂肪瘤（AML））而出现腹膜后出血。回顾了肾 AML 和肺 LAM 之间的关联。肾 AML 是肺 LAM 最常见的肺外表现。在通过腹部计算机断层扫描 (CT) 扫描进行系统检查的病例中，有 32% 至 60% 的病例发现了这种情况。建议采用后一种方法，以帮助避免肾 AML 引起的并发症。肾 AML 的治疗建议基于肿瘤大小或症状的存在。相反，患有 AML 的绝经前女性应通过高分辨率 CT 扫描检查相关肺部 LAM。由于 LAM 很可能依赖于雌激素，因此应考虑建议的几种抗雌激素疗法之一。最后，肾 AML、肺 LAM 和结节性硬化症之间存在显着重叠。因此，无论是 AML 还是 LAM，都应该积极寻找后者。 (C) 2003 年，国家肾脏基金会 (National Kidney Foundation, Inc.)
The authors report on a premenopausal female hemodialysis patient with relapsing pneumothorax, in whom the diagnosis of pulmonary lymphangioleiomyomatosis (LAM) was made. Ten years earlier, she had retroperitoneal bleeding from a kidney tumor corresponding to an angiomyolipoma (AML). The association between renal AML and pulmonary LAM is reviewed. Renal AML represents the most frequent extrapulmonary manifestation of pulmonary LAM. It is found in 32% to 60% of cases in which a systematic search with abdominal computed tomography (CT) scan is done. The latter approach is advised to help avoid complications caused by renal AML. Therapeutic recommendations for renal AML are based on tumor size or presence of symptoms. Conversely, premenopausal women presenting with AML should be investigated for associated pulmonary LAM with high-resolution CT scan. Because LAM is very likely estrogen dependent, one of the several proposed antiestrogen therapies should be considered. Finally, there is significant overlap between renal AML, pulmonary LAM, and tuberous sclerosis. The latter should therefore be actively searched for in case of either AML or LAM. (C) 2003 by the National Kidney Foundation, Inc.