Zhejiang Key Laboratory of Diagnosis & Treatment Technology On Thoracic Oncology (Lung and Esophagus), Zhejiang Cancer Hospital, Hangzhou, 310022, Zhejiang, China.
Department of Thoracic Medical Oncology, Zhejiang Cancer Hospital, Hangzhou, 310022, Zhejiang, China.
Diagn Pathol. 2024 Feb 22;19(1):37. doi: 10.1186/s13000-024-01460-x.
Primary pulmonary hyalinizing clear cell carcinoma (HCCC) is a very rare lung tumor that accounts for less than 0.09% of all primary lung tumors and has no specific epidemiology. The correct diagnosis requires imaging, laboratory, pathological, immunohistochemical, and molecular examination. The most typical feature of pulmonary HCCC is the clear cell component with clear stroma. In addition, the fusion gene EWSR1::ATF1 due to t(12;22)(q13;q12) is essential for the pathological diagnosis of pulmonary HCCC. The main treatment for pulmonary HCCC is surgery. This review focus on the pathological features, immunohistochemical examination, mutation analysis and treatment of pulmonary HCCC.
原发性肺透明细胞黏液样癌（HCCC）是一种非常罕见的肺部肿瘤，占所有原发性肺部肿瘤的比例不到 0.09%，且无特定的流行病学特征。正确的诊断需要影像学、实验室、病理学、免疫组织化学和分子检查。肺部 HCCC 最典型的特征是具有透明基质的透明细胞成分。此外，融合基因 EWSR1::ATF1 因 t(12;22)(q13;q12)而存在，对于肺部 HCCC 的病理诊断至关重要。肺部 HCCC 的主要治疗方法是手术。本文重点介绍肺部 HCCC 的病理特征、免疫组织化学检查、突变分析和治疗。
