Pulmonary adenoid cystic carcinoma: molecular characteristics and literature review

Diagnostic Pathology

Review

Ying Fan¹  Zhixin Chen²  Jiapeng Jiang²  Hongyang Lu³ 

[1] Department of Medical Oncology, Lishui Municipal Central Hospital, 323000, Lishui, Zhejiang, China;The Second Clinical Medical College, Zhejiang Chinese Medical University, 310053, Hangzhou, P.R. China;Zhejiang Key Laboratory of Diagnosis & Treatment Technology on Thoracic Oncology (lung and esophagus), Zhejiang Cancer Hospital, Hangzhou Institute of Medicine (HIM), Chinese Academy of Sciences, 310022, Hangzhou, Zhejiang, P.R. China;Department of Thoracic Medical Oncology, Zhejiang Cancer Hospital, Hangzhou Institute of Medicine (HIM), Chinese Academy of Sciences, 310022, Hangzhou, Zhejiang, P.R. China;Zhejiang Key Laboratory of Diagnosis & Treatment Technology on Thoracic Oncology (lung and esophagus), Zhejiang Cancer Hospital, Hangzhou Institute of Medicine (HIM), Chinese Academy of Sciences, 310022, Hangzhou, Zhejiang, P.R. China;Department of Thoracic Medical Oncology, Zhejiang Cancer Hospital, Hangzhou Institute of Medicine (HIM), Chinese Academy of Sciences, 310022, Hangzhou, Zhejiang, P.R. China;

关键词: PACC;    Immunohistochemistry;    Molecular characteristics;    Diagnosis;    Treatment;    Prognosis;   

DOI  :  10.1186/s13000-023-01354-4

 received in 2023-03-08, accepted in 2023-05-07,  发布年份 2023

来源: Springer

【 摘 要 】

BackgroundPulmonary adenoid cystic carcinoma (PACC) is an exceptionally rare salivary gland-type malignant neoplasm. Because of its clinical manifestations, imaging features are not different from other types of non-small cell lung cancer, which is a diagnostic challenge for most doctors.ConclusionsA review of the literature shows that high amounts of immunohistochemical (IHC) markers, such as CK7, CD117, P63, SMA, CK5/6, and S-100 are helpful for PACC diagnosis. Surgical resection is the main treatment of PACC, but treatment options for advanced PACC patients are limited and the research of molecular targeted drugs is ongoing in advanced cases not eligible for surgery. Currently, research on PACC targeted therapy mainly focuses on the exploration of v-myb avian myeloblastosis virus oncogene homolog (MYB) and its downstream target genes. In addition, median tumor mutation burden and PD-1/PD-L1 were lower in PACC, which may indicate poor efficacy of immunotherapy in PACC patients. This review focuses on the pathologic features, molecular characteristics, diagnosis, treatment and prognosis of PACC to establish a comprehensive understanding of PACC.

【 授权许可】

CC BY   
© The Author(s) 2023

【 图 表 】

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