1. Department of Medical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
2. Department of Medical Oncology, Beijing Chest Hospital, Capital Medical University & Beijing Tuberculosis and Thoracic Tumor Research Institute, Beijing 101149, China
3. Department of Comprehensive Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
4. Department of Thoracic Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
5. State Key Laboratory of Molecular Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100021, China
wangyanyifu@163.com
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History+
Received
Accepted
Published Online
2024-10-10
2025-04-23
2025-05-07
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Abstract
Primary pulmonary nuclear protein of the testis (NUT) midline carcinoma (NMC) is a rare and highly aggressive thoracic malignancy that poses significant diagnostic and therapeutic challenges in clinical practice. This tumor is characterized by its heterogeneous clinical presentations and poor prognosis, often evading accurate initial diagnosis. In this study, we present two cases of primary pulmonary NMC treated with an integrated therapeutic approach combining anti-angiogenic agents, platinum-based chemotherapy, and radiotherapy. This multimodal strategy achieved survival durations of 32 and 13 months, respectively, surpassing the currently reported median survival of advanced NMC. Through a systematic literature review of reported cases, we have summarized the currently used diagnostic methods and treatment modalities for NMC. Our findings suggest that multimodal therapy incorporating anti-angiogenic treatment may offer superior clinical outcomes compared to conventional monotherapy regimens, particularly for patients who are not eligible for surgery. This comprehensive investigation enhances our understanding of NMC management by elucidating diagnostic pitfalls through histopathological correlation and proposing an effective therapeutic combination that demonstrates improved survival outcomes. By providing valuable insights into the diagnosis and treatment of primary pulmonary NMC, we hope to contribute to the development of more effective strategies for managing this rare and aggressive malignancy.
Linyan Tian, Siyu Lei, Yaning Yang, Haiyan Xu, Chengming Liu, Yan Wang.
Anti-angiogenic therapy as a beacon of hope in the battle against pulmonary NUT midline carcinoma.
Front. Med., 2025, 19 (4) : 681-688 DOI:10.1007/s11684-025-1145-3
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