肺良性转移性平滑肌瘤临床、影像及病理学对照研究

Comparative study of clinical, imaging and pathological of pulmonary benign metastasizing leiomyoma

目的探讨肺良性转移性平滑肌瘤(Pulmonary Benign Metastasizing Leiomyoma,PBML)临床、影像及病理学特点。方法对2014年1月-2018年12月文献报道65例PBML及本院1例PBML病例的临床、影像及病理学特点进行总结、分析。结果 66例PBML患者均为女性,平均年龄(47.21±7.58)岁,14例(21.21%)患者现有子宫肌瘤,50例(75.67%)既往子宫肌瘤手术距此次发现PBML间隔平均(8.89±5.56)年。66例PBML患者中45例(68.18%)无呼吸道症状。影像学表现:54例(81.82%)表现为双肺多发类圆形结节/肿块,其中有6例(1%)部分结节/肿块伴空洞;6例(9.10%)表现为肺内单发结节/肿块;4例(6.10%)表现为粟粒性结节;2例(3.03%)表现为双肺多发囊腔样病变。66例PBML患者中,65例(98.48%)无胸腔积液及胸膜肥厚,64例(96.97%)不伴有纵隔或肺门淋巴结肿大。10例增强扫描中,8例(80.00%)伴有中度-明显强化。15例患者接受PET-CT检查,所有病灶内均无放射性浓聚。病理学特征:所有肺部病灶均为梭形细胞,结合免疫组化结果,其中雌激素受体(ER)、Desmin、SMA、孕激素受体(PR)、Ki-67<5%阳性分别为100%,100%、100%、94.29%、91.67%,HMB-45、S-100阴性100%。结论 PBML多发生于既往或现有子宫肌瘤的女性,其中绝大多数患者无肺部相关临床症状。PBML影像学多表现为双肺多发类圆形结节/肿块,CT影像表现缺乏特征性;PET-CT显示其无放射性浓聚,该点有助于与肺部恶性转移瘤相鉴别,但其确诊需要结合病理及免疫组化,其中ER、PR、SMA、Desmin阳性,有助于明确诊断。

Objective To investigate the clinical,imaging and pathological features of pulmonary benign metastasizing leiomyoma. Methods The clinical, imaging and pathological features of 65 cases PBML which published from January 2014 to December 2018 and 1 case PBML in our hospital were summarized and analyzed. Results All 66 patients with PBML were female, with an average age was(47.21±7.58) years. 14 patients(21.21%) had uterine fibroids, and the average interval of 50 patients(75.67%) from the first uterine fibroids operation was(8.89±5.56) years. 45 patients of PBML were without any respiratory symptoms. The CT features included multiple nodules/mass in bilateral lungs(54/66, 6 cases with cavitation), single nodules/mass(6/66), diffuse miliary nodules(4/66), and multiple cystic airspaces lesions in bilateral lungs(2/66). Without pleural effusion or pleural hypertrophy(65/66), without mediastinal or helix lymph node enlargement(64/66). Among 10 patients with enhanced CT scanning, 8 patients showed medium-high enhancement. 15 patients underwent PET-CT examination, and the results showed there was no hypermetabolic activity in all lesions. Pathological features were as follows: all pulmonary leisons showed as spindle cells. Combined with immunohistochemical results, the positive rate of estrogen receptor(ER), Desmin, SMA, progesterone receptor(PR), and Ki-67(+)<5% were were 100%, 100%, 100%, 94.29%, and 91.67% respectively, while the negative rate of HMB-45 and S-100 were both 100%. Conclusion PBML mostly occurs in women with previous or existing uterine leiomyoma, the vast majority of patients have no respiratory associated clinical symptoms. PBML imaging is mostly characterized by multiple circular nodules/masses in both lungs,but CT imaging without obvious imaging characteristics. PET-CT shows that there is no hypermetabolic activity,which is helpful to distinguish it from pulmonary malignant metastasis. The diagnosis needs to combine with pathology and immunohistochemistry,and positive ER,PR,SMA,and Desmin are helpful for definite diagnosis.