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目的:提高对伴有肾错构瘤的淋巴管肌瘤病(LAM)的诊治水平。方法:对2006年1月~2015年4月收治的21例伴有肾错构瘤的LAM患者的诊治情况进行分析,并复习最新文献予以讨论。结果:21例患者均为育龄期女性,散发型LAM者14例,平均年龄39.7岁,其中伴双肾多发错构瘤4例,单发10例,肾错构瘤破裂5例,4例行保守治疗成功,另1例行肾切除术;结节硬化伴发型LAM(TSC-LAM)者7例,平均年龄36.8岁,均为双肾多发错构瘤,破裂2例,均行保守治疗成功。结论:对于肾错构瘤的育龄期女性,尤其是双肾多发肾错构瘤的患者,应行胸部CT检查,以明确有无LAM;肾错构瘤是LAM的重要肺外表现之一;密切注意肿瘤破裂的可能,如果发生破裂,以保守治疗为主,对于单发肿瘤破裂,必要时可行手术治疗。
Objective: To improve the diagnosis and treatment of lymphangiomyosarcoma (LAM) associated with renal hamartoma. Methods: The diagnosis and treatment of 21 cases of LAM with renal hamartoma admitted from January 2006 to April 2015 were analyzed and the latest literatures were reviewed. Results: All the 21 patients were female of childbearing age. There were 14 cases of sporadic LAM with the average age of 39.7 years. Among them, 4 cases had multiple hamartomas in the kidneys, 10 cases were single, 5 cases were rupture of renal hamartomas, 4 cases were routine Conservative treatment was successful, and another case of nephrectomy; nodular sclerosis with concomitant LAM (TSC-LAM) in 7 cases, the average age of 36.8 years, multiple renal hamartoma, rupture in 2 cases, both conservative treatment success . CONCLUSIONS: Women of childbearing age, especially those with multiple nephrotic nephroma, should be examined by chest CT in order to find out whether there is LAM. Renal hamartoma is one of the most important extrapulmonary manifestations of LAM. Pay close attention to the possibility of tumor rupture, if the rupture, conservative treatment, single tumor rupture, if necessary, feasible surgical treatment.