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.
GU Bin, WANG Chaofu, JIN Xiaolong, YUAN Fei, ZHANG Jing, XU Haimin, REN Jingli, CHEN Xiaoyan
. Pulmonary sclerosing pneumocytoma: a clinicopathologic analysis of 23 cases[J]. Journal of Diagnostics Concepts & Practice, 2017
, 16(02)
: 188
-194
.
DOI: 10.16150/j.1671-2870.2017.02.013
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