• Liqiao Chen, Ning Zhou, Shuya Hu, Fanrong Wang, Tingting Xu, Tao Li, Yangyan Fu, Yiman Luo, and Ying Chen.
• Department of Pathology, Sichuan Mianyang 404 Hospital, Mianyang, China.
• Medicine (Baltimore). 2023 Jun 23; 102 (25): e34101e34101.

BackgroundHyalinising clear cell carcinoma (HCCC) of the lung is a rare tumor, with only 12 reported cases. To improve the differential diagnosis, the aim of this study was to clarify the clinicopathological characteristics, immunophenotype, and molecular characteristics of HCCC of the lung and relate these to prognosis.MethodsSections of HCCC of the lung were collected from a patient for pathological observation, immunohistochemistry, histochemistry, and fluorescence in situ hybridization; the clinical, pathological, and molecular characteristics were compared with those reported in the literature.ResultsThe tumor had a well-demarcated border nodule with a maximal diameter of 2.5 cm. Microscopic findings showed either clear or eosinophilic cytoplasm in the tumor cells. Growth was predominantly in the sheets, nests, and trabeculae in a background of hyalinised, fibrotic stroma, and mucus degeneration. Immunohistochemistry showed that the tumor cells expressed cytokeratin 7, P63, P40, CK5/6, Pan Cytokeratin (PCK), and epithelial membrane antigen, whereas they were negative for thyroid transcription factor-1, napsin A, CD10, vimentin, and smooth muscle actin. The Ki67 proliferation index was 5%. The tumor was positive for both period acid-Schiff (PAS) and Alcian blue-PAS, with a small amount of mucus staining positive for PAS-diastase. Fluorescence in situ hybridization revealed Ewing sarcoma breakpoint region 1 rearrangement and Ewing sarcoma breakpoint region 1-activating transcription factor 1 fusion.ConclusionsHCCC is a low-grade carcinoma with excellent prognosis. Tumour necrosis may be a potential risk factor for recurrence and metastasis. Our review of reported cases suggests that regional lymph node dissection combined with lobectomy is a safer treatment than only lobectomy for HCCC of the lung.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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