肺淋巴管平滑肌瘤病伴右肾血管平滑肌脂肪瘤临床病理观察  

Pulmonary lymphangioleiomyomatosis accompanied with renal angiomyolipoma:report of a case and literature review

作　　者：戴芳[1] 李学锋[1] 付红梅[2] 李涛[1] 王丽[1] 杨丽琳[1] 王春燕[2] 蔡琳[1] 杨举伦[1] 

机构地区：[1]成都军区昆明总医院病理科,昆明650032 [2]昆明医学院第一附属医院病理科,昆明650032

出　　处：《诊断病理学杂志》2011年第4期268-271,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨肺淋巴管平滑肌瘤病(PLAM)伴右肾血管平滑肌脂肪瘤(AML)的临床特点、影像特征、病理学和预后,对比两种疾病的异同点。方法回顾性分析1例PLAM伴AML患者的临床、影像和病理资料,并复习文献。结果 PLAM主要发生于育龄期女性,临床症状无特异性,以咳嗽、胸闷、气短和呼吸困难为主;病理检查示肺组织内不同成熟度的平滑肌细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生,肺实质呈囊性变。免疫组化染色示增生的平滑肌细胞SMA、HMB45、desmin和PR均(+),ER(-);瘤样淋巴管D2-40(+)。肾AML由比例不等的脂肪组织、梭形平滑肌细胞和异常的厚壁血管3种成分构成,梭形或上皮样的平滑肌细胞SMA、HMB45和desmin(+)。结论 PLAM伴肾AML少见,病因不清,同属于具有血管周上皮样细胞分化的肿瘤。PLAM和肾AML同时出现,应考虑是否与结节性硬化症有关。Objective To investigate the clinicopathological features and prognosis of pulmonary lymphangioleiomyomatosis（PLAM） accompanying with renal angiomyolipoma（AML） to emphasize the diagnosis and differential diagnosis of the disease.Methods By routine histological and immunohistochemical（IHC） methods,the clinicopathological features and prognosis were analyzed in 1 case of PLAM and the related literatures were reviewed.Results PLAM occured almost exclusively in women of reproductive age.The main clinical manifestations of PLAM were nonspecific,including cough,suffocated chest,short breathing and dyspnea,pneumothorax and chylothorax.The pathological features of PLAM were abnormal hyperplasia of smooth muscle cells（PLAM cells） surrounding alveolar walls,bronchioles,lymphatics and blood vessels leading to airflow obstruction and replacement of the lung parenchyma by cysts.Immunohistochemical staining showed that the characteristic smooth muscle cells were positive for SMA,HMB-45,desmin,estrogen receptor,and negative for progesterone receptor.Renal AML was composed of different proportions of blood vessels,smooth muscle cells and adipose tissues.Immunohistochemical staining showed that smooth muscle cells were positive for SMA,HMB-45,and desmin.Conclusion PLAM accompanied renal AML is a rare disease of unknown etiology,which belongs to perivasular epithelioid cell tumor（PEComa）.The coexisting of PLAM and renal ALM may be associated with tuberous sclerosis complex.

关 键 词：肺淋巴管平滑肌瘤病 血管平滑肌脂肪瘤 结节性硬化症 

分 类 号：R734.2[医药卫生—肿瘤]