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NapsinA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 characteristics and pathological features of pulmonary sclerosing pneumocytoma（PSP）. Methods: A total of 23 cases of PSP were enrolled,and the clinical and pathologic features, imaging findings and immunophenotype were collected and analyzed retrospectively. The associated literature was reviewed. Results: PSP was more common in adult females than male, most patients were symptom-free（13/23,56.5%）. Of the 23 cases, 3 cases（3/23, 13.0%）were preoperatively diagnosed as PSP by imaging, and 13 cases were diagnosed as PSP（13/23, 56.5%）by frozen section examination at operation and the diagnostic rate was low. Gross examination revealed that PSP presented with a well-circumscribed mass, with no capsule or pseudocapsule,and a grey to tan color cut surface, 7 cases had focal dark red hemorrhage area. Microscopic examination revealed that PSP showed four histological patterns (solid, papillary, hemorrhagic and sclerotic) and two types of tumor cells（cuboidal surface cells and round or polygon stromal cells）. For immunophenotype, TTF-1 was strongly expressed on both surface cells and stromal cells. AE1/AE3, CAM5.2 and EMA were strongly expressed on the cell membrane and cytoplasm of surface cells, while weakly expressed on the cell cytoplasm of only a minority of stromal cells. NapsinA and SP-A were only expressed on surface cells, and Vimentin on stromal cells. All the 23 patients were followed up from 4 to 50 months, one case was found having contralateral pulmonary nodule of 1.5 cm on CT at 9 months after surgery, and radiographic surveillance was to be continued. The other 22 patients had no recurrence or metastasis. Conclusions: PSP lacks special clinical and imaging manifestations and having a high misdiagnosis rate of frozen section pathology examination. The pathological diagnosis depends on the morphologic characteristics of paraffin-embedded tissue sections and immunohistochemical staining.
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