30例肺硬化性肺细胞瘤临床病理特征分析

Clinicopathological analysis of 30 cases pulmonary sclerosing pneumocytoma

目的 总结肺硬化性肺细胞瘤(pulmonary sclerosing pneumocytoma, PSP)的临床病理特征,提高对该疾病的病理形态学、免疫组化特点的认识。方法 收集2018年1月1日至2020年12月31日辽宁省肿瘤医院收治的30例PSP患者的临床病理资料。所有患者均接受外科手术治疗及随访。免疫组化观察CK、Vimentin、TTF-1、EMA、PR、Ki-67表达情况。总结PSP的临床病理特征。结果 30例患者中女性27例,男性3例,女性占绝对优势(女∶男=9∶1),年龄30~65岁,中位年龄55.5岁。左肺上叶3例,左肺下叶8例,右肺上叶2例,右肺下叶13例,右肺中叶4例。伴淋巴结转移1例,合并同侧肺叶浸润性腺癌4例。影像学检查大多提示为边界清晰的类圆形结节。显微镜下组织学形态均可见实性区、乳头状区、出血区和硬化区,由两种肿瘤细胞(立方样细胞和间质圆形细胞)构成,所有病例免疫组化表达模式一致:两种细胞都表达EMA和TTF-1,立方样细胞表达CK,间质圆形细胞表达TTF-1、Vimentin、PR,Ki-67低表达(<3%)。结论 PSP为临床罕见的肺部良性肿瘤,多发生于中年女性,临床特点及影像学检查缺乏特异性,术中快速冰冻病理诊断容易误诊,掌握该疾病基本病理组织形态学基础及免疫组化组合应用具有辅助诊断作用。

Objective To analyze the clinicopathological characteristics of pulmonary sclerosing pneumocytoma(PSP), and to improve the understanding of the histopathologic and immunohistochemical characteristics of the disease. Methods The clinicopathological data of 30 patients with PSP admitted to Liaoning Cancer Hospital from January 1, 2018 to December 31, 2020 were collected. All patients received surgical treatment and follow-up. The expressions of CK, Vimentin, TTF-1, EMA, PR and Ki-67 were examined by immunohistochemistry. The clinicopathological features of PSP were analyzed. Results Among the 30 patients, 27 were female and 3 were male with a female predominance(F∶M ratio 9∶1). The age ranged from 30 to 65 years old, with a median age of 55.5 years. There were 3 cases in the left upper lobe, 8 cases in the left lower lobe, 2 cases in the right upper lobe, 13 cases in the right lower lobe, and 4 cases in the right middle lobe. There was 1 case with lymph node metastasis and 4 cases with invasive adenocarcinoma in the ipsilateral lobe. Imaging studies revealed mostly round nodules with clear border. Microscopic examination showed a variation of solid areas, papillary areas, hemorrhagic areas and sclerotic areas, which were composed of two types of tumor cells(cubic cells and stromal round cells). All cases had similar immunohistochemical expression patterns: both cell types expressed EMA and TTF-1, cuboid cells expressed CK, and stromal round cells expressed TTF-1, Vimentin and PR. Ki-67 immunohistochemical study revealed a low proliferation rate(<3%). Conclusion Pulmonary sclerosing pneumocytoma is a rare benign tumor of the lung, which mostly occurs in middle-aged women. The clinical features and imaging examinations lack specificity. The rapid intraoperative frozen sections are easy to be misdiagnosed. Understanding the basic histopathologic morphology of the disease and the combined application of immunohistochemistry can assist diagnosis.