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目的:探讨肺硬化性肺细胞瘤(pulmonary sclerosing pneumocytoma,PSP)患者的临床表现、影像学特点和病理学诊断。方法:收集23例PSP患者的临床病理资料,分析其临床表现、影像学特点、病理学特征以及免疫表型,并复习相关文献进行探讨。结果:PSP多见于成年女性,多数患者无症状(13/23,56.5%)。23例患者中,3例(3/23,13.0%)术前经影像学诊断为PSP,13例(13/23,56.5%)于术中经冷冻病理检查诊断为PSP,诊断准确率均较低。大体检查可见,肿块边界清晰,无包膜或有假包膜,切面灰白至灰褐色,7例有出血,出血区呈暗红色。光镜检查可见实性、乳头状、血管瘤样和间质硬化4种结构,表面立方形细胞及间质圆形或多角形细胞2种细胞。免疫表型为,TTF-1在表面细胞及间质细胞均强表达,AE1/AE3、CAM5.2、EMA表面细胞胞膜及细胞质强表达,间质细胞部分呈细胞质弱表达,仅在表面细胞表达Napsin A及SP-A,仅在间质细胞表达Vimentin。23例患者均获得随访,随访时间为4~50个月,其中1例术后9个月发现对侧肺叶有直径为1.5 cm的小结节,未行手术,仍在随访中,其余均无复发及转移。结论:PSP患者缺少特征性的临床和影像学表现;术中冷冻病理检查误诊率高,确诊需要进行石蜡组织形态学观察和免疫组织化学标记检测。
Objective: To investigate the clinical manifestations, imaging features and pathological diagnosis of patients with pulmonary sclerosing pneumocytoma (PSP). Methods: The clinical and pathological data of 23 patients with PSP were collected, and their clinical manifestations, imaging features, pathological features and immunophenotypes were analyzed. Relevant literatures were reviewed. Results: PSP more common in adult women, the majority of patients without symptoms (13 / 23,56.5%). Three of the 23 patients (3 / 23,13.0%) were preoperatively diagnosed as PSP by imaging and 13 (13 / 23,56.5%) were diagnosed as PSP by intraoperative cryopathology, the diagnostic accuracy was higher low. Gross examination shows that the mass of clear boundary, no capsule or a pseudocapsule, section gray to brown, 7 cases of bleeding, bleeding area was dark red. Light microscopy showed solidity, papillary, hemangioid and interstitial sclerosis 4 kinds of structures, the surface of cubic cells and interstitial round or polygonal cells 2 kinds of cells. The immunophenotype was that TTF-1 was strongly expressed on the surface cells and stromal cells, the membrane and cytoplasm of AE1 / AE3, CAM5.2 and EMA were strongly expressed, and the interstitial cells were weakly expressed on the cytoplasm. Expressing Napsin A and SP-A, Vimentin is expressed only in stromal cells. Twenty-three patients were followed up for 4 to 50 months. One case had a small nodule 1.5 cm in diameter at 9 months after operation. All the patients were still under follow-up without surgery. Recurrence and metastasis. Conclusion: PSP patients lack of characteristic clinical and imaging findings; intraoperative frozen pathological examination misdiagnosis rate, the diagnosis required paraffin morphology and immunohistochemical detection.