肺良性转移性平滑肌瘤的组织发生和临床病理学关系

Clinicopathology and Histogenesis of Pulmonary Benign Metastasizing Leiomyoma

目的探讨肺良性转移性平滑肌瘤(PBML)的组织发生和生物学性质以及与子宫平滑肌瘤的关系。方法对3例子宫平滑肌瘤术后伴发PBML(BML组)的肺活检组织进行光学显微镜和免疫组织化学研究、临床病理资料分析和治疗后随访,与3例原发性肺纤维平滑肌瘤性错构瘤(PFH)(FH组)进行对照。结果BML组3例为女性患者,均有子宫平滑肌瘤手术史,子宫切除至出现肺部病灶平均10.6年。胸部CT显示双肺多发性结节影,边缘清楚。光学显微镜观察:两组瘤细胞均呈梭形,弥漫排列,其间有柱状上皮构成的腺样结构,分化成熟。免疫组织化学:两组梭形瘤细胞均表达波形蛋白、结蛋白、平滑肌肌动蛋白、雌、孕激素受体,不表达广谱角蛋白、上皮膜抗原、S-100、神经元特异性烯醇化酶和嗜铬素A;两组腺样结构均表达甲状腺转录因子-1、表面活性蛋白A、B、广谱角蛋白;两组表达差异无统计学意义(P>0.05)。随访6月～4年不等,肺部病灶进展缓慢。结论PBML与PFH的瘤细胞形态和免疫表型相似,梭形瘤细胞似分化成熟的平滑肌细胞和肌纤维母细胞,腺样结构起源于呼吸上皮,提示BML可能是原发于肺的多发性纤维平滑肌瘤性错构瘤。

Objective To investigate the biological features and histogenesis of pulmonary benign metastasizing leiomyoma （PBML）, and to reveal the realationship between PBML and fibromyoma uteri. Methods The clinicopathological features and immunohistochemistry of 3 patients with PBML and 3 cases of pulmonary fibroleiomyomatous hamartoma（PFH） were reviewed and compared, respectively. Followed-up data were also obtained. Results The 3 female patients in PBML group had fibromyoma uteri history with average time of 10.6 year from hysterectomia to the appearance of PBML lesions. Chest CT scan showed that multiple well defined nodules in the both lobes of the lungs. The tumors two groups exhibited similar in histopathological and immunohistochemistric features. The optical observation showed that the tumor cells were in fusiform shape, and in scattered layout. The well differentiated adenoid columnar epithelium were observed in the tumors.Immunohistochemistry showed the fusiform shaped cells expressed vimentin, desmin,α-actin, estrogen receptor and progesterone receptor,while didn’t express cytokeratin expression, epithelial membrane antigen, S-100, neurospecific enolase and chromogranin A Meanwhile the surfactant proteins adenoid structures also expressed thyroid transcription factor-1, surfactant proteins A, surfactant proteins B and pancytokeratin. There was not significant difference in the expression of these markers in both tumors（P〉0.05）. The lesions of PBML developed slowly during the 6 months to 4 years follow up. Conclusion The PBML resembled PFH showed the similar histopathological features and immunophenotypes. Fusiform-shaped cells of PBML were similar to the well developed smooth muscle cells and myofibroblasts. Adenoid structures originated from respiratory epithelium. The results suggested that the tumor maybe originate from lung multiple fibroleiomyomatous hamartoma.