Pulmonary sclerosing pneumocytoma: a clinicopathologic analysis of 13 cases_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

HUANG Hong ¹ , CHEN Xiaoli ¹ , CHEN Xin ² ,  YANG Jun ¹

1. Department of Pathology, the Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710004, P. R. China;
2. Department of Radiology, the Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi 710004, P. R. China;

Corresponding?author：

YANG Jun, Email: yangjundr@163.com

Keywords：

Pulmonary sclerosing pneumocytoma; Clinicopathology; Frozen section pathology examination; Differential diagnosis.

DOI：

10.7507/1671-6205.202104037

Video：

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Objective To investigate the clinicopathologic features, diagnosis and differential diagnosis of pulmonary selerosing pneumocytoma (PSP). Methods A total of 13 cases of PSP were enrolled, and the clinical and imaging findings, pathologic features, and immunophenotype were collected and analyzed, with review of the literatures. Results Thirteen patients were all female, aged from 27 to 69 years old by first discovered, the average age was 53 years old. The maximum diameter ranged from 0.8 - 6 cm. It was mainly discovered accidentally by physical examination. According to the CT findings of 13 cases, all lesions were round or round-like, with a well-circumscribed mass. The “welt vessel sign” was suggestive to the diagnosis of PSP. Microscopically, the tumor was composed of two types of cells (surface epithelial cells and round mesenchymal cells), and four histological patterns (papillary, solid, hemorrhagic and sclerotic zone). Immuophenotype: thyroid transcription factor-1 (TTF-1) and epithelial membrane antigen were expressed on both epithelial cells and mesenchymal cells. Pan cytokeratin (PCK) and cytokeratin 7 were only expressed on epithelial cells, and vimentin on mesenchymal cells. Conclusion PSP is a rare lung benign tumor, preoperative and intraoperative freezing diagnosis are difficult, the diagnosis depends on the morphologic characteristics of paraffin-embedded tissue sections and immunohistochemical staining. The identification of mesenchymal cells with TTF-1 positive and PCK negative is the key to PSP diagnosis.

Citation： HUANG Hong, CHEN Xiaoli, CHEN Xin, YANG Jun. Pulmonary sclerosing pneumocytoma: a clinicopathologic analysis of 13 cases. Chinese Journal of Respiratory and Critical Care Medicine, 2021, 20(12): 862-869. doi: 10.7507/1671-6205.202104037 Copy

1.	于洋濤, 胡偉, 劉金耿, 等. 60例硬化性肺細胞瘤診治分析. 現代腫瘤醫學, 2019, 27(19): 3443-3446.
2.	Yang CH, Lee LY. Pulmonary sclerosing pneumocytoma remains a diagnostic challenge using frozen sections: a clinicopathological analysis of 59 cases. Histopathology, 2018, 72: 500-508.
3.	張杰東, 吳鴻雁, 孟凡青, 等. 伴淋巴結轉移的硬化性肺細胞瘤1例. 臨床與實驗病理學雜志, 2021, (11): 1398-1399.
4.	Liebow AA, Hubbell DS. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer, 1956, 9: 53-75.
5.	Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, et al. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol, 2000, 24: 906-916.
6.	Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification. J Thorac Oncol, 2015, 10(9): 1243-1260.
7.	Bara A, Adham I, Daaboul O, et al. Sclerosing pneumocytoma in a 1-year-old girl presenting with massive hemoptysis: a case report. Ann Med Surg (Lond), 2021, 62: 49-52.
8.	Zhu J. Analysis of the clinical differentiation of pulmonary sclerosing pneumocytoma and lung cancer. J Thorac Dis, 2017, 9(9): 2974-2981.
9.	曹輝, 田成斌, 史曉光. 28例孤立性硬化性肺泡細胞瘤CT影像表現. 臨床肺科雜志, 2018, 23(11): 173-175.
10.	朱靚, 王旭榮, 邱乾德. 肺硬化性肺泡細胞瘤CT表現. 醫學影像學雜志, 2019, 29(10): 1717-1720.
11.	Khanna A, Alshabani K, Mukhopadhyay S, et al. Sclerosing pneumocytoma: Case report of a rare endobronchial presentation. Medicine (Baltimore), 2019, 98(15): e15038.
12.	Teng X, Teng X. First report of pulmonary sclerosing pneomucytoma with malignant transformation in both cuboidal surface cells and stromal round cells: a case report. BMC Cancer, 2019, 19(1): 1154.
13.	Gao Q, Zhou J, Zheng Y, et al. Clinical and histopathological features of pulmonary sclerosing pneumocytoma with dense spindle stromal cells and lymph node metastasis. Histopathology, 2020, 77(5): 718-727.
14.	顧斌, 王朝夫, 金曉龍, 等. 肺硬化性肺細胞瘤23例臨床病理分析. 診斷學理論與實踐, 2017, 16(2): 188-194.
15.	Lovrenski A, Vasilijevi? M, Panjkovi? M, et al. Sclerosing pneumocytoma: a ten-year experience at a Western Balkan University Hospital. Medicina (Kaunas), 2019, 55(2): 27.
16.	Jung SH, Kim MS, Lee SH, et al. Whole-exome sequencing identifies recurrent AKT1 mutations in sclerosing hemangioma of lung. Proc Natl Acad Sci USA, 2016, 113(38): 10672-10677.
17.	Fan XS, Lin L, Wang JJ, et al. Genome profile in a extremely rare case of pulmonary sclerosing pneumocytoma presenting with diffusely-scattered nodules in the right lung. Cancer Biol Ther, 2018, 19(1): 13-19.
18.	劉小靜, 黃志豪, 張建勇. 硬化性肺泡細胞瘤35例臨床特征分析. 中國肺癌雜志, 2020, 23(12): 1049-1058.
19.	Kim MK, Jang SJ, Kim YH, et al. Bone metastasis in pulmonary sclerosing hemangioma. Korean J Intern Med, 2015, 30(6): 928-930.
20.	Sakai T, Miyoshi T, Umemura S, et al. Large pulmonary sclerosing pneumocytoma with massive necrosis and vascular invasion: a case report. Oxf Med Case Reports, 2019, 2019(7): omz066.

1. 于洋濤, 胡偉, 劉金耿, 等. 60例硬化性肺細胞瘤診治分析. 現代腫瘤醫學, 2019, 27(19): 3443-3446.
2. Yang CH, Lee LY. Pulmonary sclerosing pneumocytoma remains a diagnostic challenge using frozen sections: a clinicopathological analysis of 59 cases. Histopathology, 2018, 72: 500-508.
3. 張杰東, 吳鴻雁, 孟凡青, 等. 伴淋巴結轉移的硬化性肺細胞瘤1例. 臨床與實驗病理學雜志, 2021, (11): 1398-1399.
4. Liebow AA, Hubbell DS. Sclerosing hemangioma (histiocytoma, xanthoma) of the lung. Cancer, 1956, 9: 53-75.
5. Devouassoux-Shisheboran M, Hayashi T, Linnoila RI, et al. A clinicopathologic study of 100 cases of pulmonary sclerosing hemangioma with immunohistochemical studies: TTF-1 is expressed in both round and surface cells, suggesting an origin from primitive respiratory epithelium. Am J Surg Pathol, 2000, 24: 906-916.
6. Travis WD, Brambilla E, Nicholson AG, et al. The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification. J Thorac Oncol, 2015, 10(9): 1243-1260.
7. Bara A, Adham I, Daaboul O, et al. Sclerosing pneumocytoma in a 1-year-old girl presenting with massive hemoptysis: a case report. Ann Med Surg (Lond), 2021, 62: 49-52.
8. Zhu J. Analysis of the clinical differentiation of pulmonary sclerosing pneumocytoma and lung cancer. J Thorac Dis, 2017, 9(9): 2974-2981.
9. 曹輝, 田成斌, 史曉光. 28例孤立性硬化性肺泡細胞瘤CT影像表現. 臨床肺科雜志, 2018, 23(11): 173-175.
10. 朱靚, 王旭榮, 邱乾德. 肺硬化性肺泡細胞瘤CT表現. 醫學影像學雜志, 2019, 29(10): 1717-1720.
11. Khanna A, Alshabani K, Mukhopadhyay S, et al. Sclerosing pneumocytoma: Case report of a rare endobronchial presentation. Medicine (Baltimore), 2019, 98(15): e15038.
12. Teng X, Teng X. First report of pulmonary sclerosing pneomucytoma with malignant transformation in both cuboidal surface cells and stromal round cells: a case report. BMC Cancer, 2019, 19(1): 1154.
13. Gao Q, Zhou J, Zheng Y, et al. Clinical and histopathological features of pulmonary sclerosing pneumocytoma with dense spindle stromal cells and lymph node metastasis. Histopathology, 2020, 77(5): 718-727.
14. 顧斌, 王朝夫, 金曉龍, 等. 肺硬化性肺細胞瘤23例臨床病理分析. 診斷學理論與實踐, 2017, 16(2): 188-194.
15. Lovrenski A, Vasilijevi? M, Panjkovi? M, et al. Sclerosing pneumocytoma: a ten-year experience at a Western Balkan University Hospital. Medicina (Kaunas), 2019, 55(2): 27.
16. Jung SH, Kim MS, Lee SH, et al. Whole-exome sequencing identifies recurrent AKT1 mutations in sclerosing hemangioma of lung. Proc Natl Acad Sci USA, 2016, 113(38): 10672-10677.
17. Fan XS, Lin L, Wang JJ, et al. Genome profile in a extremely rare case of pulmonary sclerosing pneumocytoma presenting with diffusely-scattered nodules in the right lung. Cancer Biol Ther, 2018, 19(1): 13-19.
18. 劉小靜, 黃志豪, 張建勇. 硬化性肺泡細胞瘤35例臨床特征分析. 中國肺癌雜志, 2020, 23(12): 1049-1058.
19. Kim MK, Jang SJ, Kim YH, et al. Bone metastasis in pulmonary sclerosing hemangioma. Korean J Intern Med, 2015, 30(6): 928-930.
20. Sakai T, Miyoshi T, Umemura S, et al. Large pulmonary sclerosing pneumocytoma with massive necrosis and vascular invasion: a case report. Oxf Med Case Reports, 2019, 2019(7): omz066.

Chinese Journal of Respiratory and Critical Care Medicine

Pulmonary sclerosing pneumocytoma: a clinicopathologic analysis of 13 cases

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