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.
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http://dx.doi.org/10.1186/s13000-024-01460-x | DOI Listing |
Front Oncol
December 2024
Pathology Department, Affiliated Hospital of Zunyi Medical University, Zunyi, Guizhou, China.
Background: Primary pulmonary hyalinizing clear cell carcinoma (HCCC) is a rare type of primary salivary gland-type tumor of the lung. HCCC is characterized by unique pathological features, including nests, cords, or trabeculae of clear or eosinophilic tumor cells infiltrating a mucinous or hyalinized stroma. Additional analyses of this carcinoma have revealed positive epithelial markers via immunophenotyping and gene translocation through genetic testing.
View Article and Find Full Text PDFDiagn Pathol
November 2024
Department of Pathology, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Background: Primary pulmonary hyalinizing clear cell carcinoma (HCCC) is an exceedingly rare tumor with unique clinicopathological features, posing major diagnostic challenges.
Case Presentation: We present a case of a 74-year-old woman with a lung nodule incidentally detected in the right middle lobe (RML) through F-fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT) imaging. Through comprehensive evaluations by thoracic surgeons, she underwent video-assisted thoracic surgery of RML lobectomy to excise the lung nodule.
Zhonghua Bing Li Xue Za Zhi
November 2024
Department of Pathology, Shanghai Chest Hospital/Chest Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200030, China.
To investigate the clinicopathological features and differential diagnosis of primary pulmonary hyalinizing clear cell carcinoma (HCCC), as well as its diagnostic pitfalls in assessing biopsy specimens. Five cases of primary pulmonary HCCC diagnosed in the Department of Pathology, Shanghai Chest Hospital, Shanghai, China from August 2019 to December 2023 were collected. The clinicopathological characteristics, immunohistochemistry, and the EWSR1 gene related translocation and fusion were summarized, and relevant literature was reviewed.
View Article and Find Full Text PDFPrague Med Rep
May 2024
Department of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, USA.
An 82-year-old woman with COPD presented to the emergency department with cough, increasing sputum production, wheezing, and worsening shortness of breath for two weeks. On imaging studies, the patient was found to have a right upper lobe spiculated nodule and an endobronchial lesion with near total occlusion of the right lower lobe bronchus with sub-segmental atelectasis. Bronchoscopy with EBUS-TBNA of subcarinal and right hilar lymph nodes revealed lung cancer with clear cell phenotype.
View Article and Find Full Text PDFDiagn Pathol
February 2024
Zhejiang Key Laboratory of Diagnosis & Treatment Technology On Thoracic Oncology (Lung and Esophagus), Zhejiang Cancer Hospital, Hangzhou, 310022, Zhejiang, China.
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