淋巴管肌瘤病并肾血管平滑肌脂肪瘤41例临床回顾研究  

Clinical retrospective analysis：41 cases of lymphangioleiomyomatosis complicated with renal angiomyolipomas

作　　者：刘杰[1] 钟南山[1] 陈荣昌[1] 李时悦[1] 彭雯[1] 顾为丽[1] 张璐[1] 顾莹莹[2] 

机构地区：[1]广州医科大学附属第一医院呼吸内科广州呼吸疾病研究所呼吸疾病国家重点实验室呼吸疾病国家临床研究中心,510120 [2]广州医科大学附属第一医院病理科,510120

出　　处：《国际呼吸杂志》2017年第22期1720-1726,共7页International Journal of Respiration

基　　金：国家重点研发计划（2016YFC0901502）;广东省医学科学技术研究基金项目（A2015018）

摘　　要：目的加强对淋巴管肌瘤病（LAM）并肾血管平滑肌脂肪瘤（AML）临床、影像、病理特点的认识，提高广大临床医师对该病的诊治水平，减少误诊、漏诊。方法对2011—2017年广州呼吸疾病研究所收治的41例LAM并肾AML患者的临床特征、影像学、病理资料进行回顾性分析。结果结节性硬化症（TSC）-AML组患者10例，平均年龄39．8岁，包括血尿6例，腰痛5例，腹部肿块3例；肾AML肿瘤数量均大于3个，肿瘤最大直径20cm；均为双侧病灶。LAM—AML组患者31例，平均年龄37．5岁，包括血尿8例，腰痛6例，腹部肿块3例；肾AML肿瘤数量l～3个有17例（54．8%），肾AML肿瘤最大直径≤3cm22例（71．0oA），肿瘤最大直径18cm；单侧肾AML病灶患者19例，双侧肾AML病灶患者12例。2组患者胸部CT均表现为双肺弥漫性囊性变并多发肺大疱；腹部增强CT显示单侧或双侧多发肾AML，其中巨大。肾AML共9例。典型肺活检病理表现为特征性肺部囊性变及多发不成熟平滑肌梭形细胞和血管周上皮细胞异常增生；免疫组织化学：SMA（＋），HMB45（＋），ER（＋），PR（＋）。典型肾脏病理表现为多发不成熟平滑肌细胞和血管周上皮细胞异常增生，脂肪细胞蓄积；免疫组织化学：SMA（＋），HMB45（＋）。TSC—AML组和LAM—AML组血清血管内皮生长因子D水平分别为2030．9ng／L（IQR：1243．1～3124．4ng／L）和1592．9ng／L（IQR：845．2～1904．9ng／L），显著高于健康对照组[268．1ng／L（IQR：190．8～364．1ng／L），P值均〈O．001]。结论LAM典型肾脏受累表现为肾AML，腹部增强CT、血清血管内皮生长因子D和肾活检有助于诊断。Objective To improve the recognization of clinical, radiological and pathological characteristics in lymphangioleiomyomatosis （LAM） complicated with renal angiomyolipomas （AML）, and to decrease misdiagnosis and missed diagnosis. Methods The clinical , radiologicaI and pathological data of 41 patients of LAM complicated with renal AML in Guangzhou Institute of Respiratory Disease from 2011 to 2017 were retrospectively analyzed. Results All patients were divided into two groups： ten cases in tuberous sclerosis complex （TSC）-AML group and 31 cases in LAM AML group. In TSC-AMI. group, patients were all female and their mean age was 39.8 years old. Clinical manifestations included hematuria （six cases）, lumbago （five cases）, abdominal mass （three cases）. All individuals had bilateral leisions and over three tumors. Maximum length of tumor was 20 cm. In I.AM AML group, patients were also female and their mean age was 37.5 years old. Clinical manifestations included hematuria （eight cases）,lumbago （six cases）, abdominal mass （three cases）. 12 cases had bilateral leisions and 19 cases had unilateral leisions. Quantity of tumor was 1-3 in 17 cases （54.8%）.There were 22 patients （71.0%） whose length of tumor was less than 3 cm. Maximum length of tumor was 18 cm. CT scans of chest in all patients indicated diffuse cystic lung. Enhanced CT scans of abdomen in all patients indentified multiple renal AML, including nine cases with enormous AML. Typical pathological features of lung included cystic change of lung and abnormal proliferation of multiple immature smooth muscle spindle cells and perivascular epithelial cells. Typical immunohistochemisty of lung biopsy included SMA （＋）, HMB45 （ ＋ ）, ER（＋ ）, and PR（＋ ）. Typical pathological manifestinations of kidney included fat cell accumulation and abnormal proliferation of multiple immature smooth muscle cells and perivascular epithelial ceils. Immunohistochemisty of kidney biopsy included SMA （q-） and HMB4

关 键 词：淋巴管肌瘤病 肺 血管平滑肌脂肪瘤 肾 

分 类 号：R733.1[医药卫生—肿瘤] R737.11[医药卫生—临床医学]