HOW COMMON ARE RENAL ANGIOMYOLIPOMAS IN PATIENTS WITH PULMONARY LYMPHANGIOMYOMATOSIS
HOW COMMON ARE RENAL ANGIOMYOLIPOMAS IN PATIENTS WITH PULMONARY LYMPHANGIOMYOMATOSIS
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DOI：
10.1164/ajrccm.152.6.8520787
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发表时间：
1995-12-01
影响因子：
24.7
通讯作者：
LYNCH, DA
中科院分区：
文献类型：
--
作者：
BERNSTEIN, SM;NEWELL, JD;LYNCH, DA
在病例报道中，肺淋巴管肌瘤病与肾血管肌脂肪瘤有关，但这种相关性的流行还没有很好的文献记录。本研究的目的是确定一系列肺淋巴管肌瘤病患者中肾血管肌脂肪瘤的发生率。至1994年间，连续收治肺淋巴管肌瘤病患者18例。在这些患者中，一名患者被排除在外，因为她没有接受腹部计算机断层扫描(CT)。我们发现17例肺淋巴管肌瘤病患者中有8例(47%)有肾血管肌脂肪瘤。这些都是在手术或腹部CT扫描中发现的，因此，肾血管肌脂肪瘤常与肺淋巴管肌瘤病相关。因此，早期通过腹部CT发现肾血管平滑肌脂肪瘤可能是重要的，因为直径大于4厘米的病变可能会增加与肿瘤生长或出血相关的并发症的风险。通过超声或CT扫描进行连续随访在识别和监测高危患者方面具有重要意义。对于有明显生长或其他并发症(如出血)的肿瘤患者，可考虑预防性治疗(部分或全部肾切除)。
Pulmonary lymphangiomyomatosis has been associated with renal angiomyolipoma in case reports, but the prevalence of this association has not been well documented. The objective of this study was to determine the frequency of renal angiomyolipoma in a series of subjects with pulmonary lymphangiomyomatosis. Eighteen consecutive patients with pulmonary lymphangiomyomatosis were seen at a single institution between 1989 and 1994. Of these, one patient was excluded because she did not have an abdominal computed tomographic (CT) scan. We found eight out of 17 (47%) patients with pulmonary lymphangiomyomatosis to have renal angiomyolipomas. These were found either at surgery or on abdominal CT scanning, Thus, renal angiomyolipomas occur commonly in association with pulmonary lymphangiomyomatosis. Consequently, the early detection of renal angiomyolipoma by abdominal CT may be important, because lesions with dimensions larger than 4 cm may present an increased risk for complications related to tumor growth or hemorrhage. Serial follow-up by ultrasonography or CT scanning is important in identifying and monitoring high-risk patients. Prophylactic treatment (partial or total nephrectomy) may be considered for patients with tumors that show significant growth or other complications, such as hemorrhage.