Kartagener syndrome with lung cancer and mediastinal tumor
Masafumi Horie1) Hidenori Arai2) Satoshi Noguchi1) Masaru Suzuki1) Yoshio Sakamoto1) Teruaki Oka3)
1)Department of Respiratory Medicine, Kanto Central Hospital 2)Department of Internal Medicine, School of Medicine, Teikyo University 3)Department of Pathology, Kanto Central Hospital
A 71-year-old man was admitted to Kanto Central Hospital with hemoptysis. He had had chronic sinusitis and deafness since childhood. Situs inversus, bronchiectasia, and diffuse panbronchiolitis had been also diagnosed at the age of 59. Chest computed tomography demonstrated a 5-cm mass in the anterior mediastinum as well as a 4-cm mass in the upper lobe of the right lung. A transbronchial lung biopsy of the right lung tumor revealed squamous cell carcinoma. Electron microscopic examination of the bronchial epithelial cilia revealed a total defect of both inner and outer dynein arms, leading to a diagnosis of primary ciliary dyskinesia. Biopsy of the mediastinal tumor was not performed. After concurrent chemoradiation therapy, the lung cancer decreased in size partial remission (PR) and the mediastinal tumor disappeared complete remission (CR). Later, a cavity formed in the tumor, where a Pseudomonas aeruginosa infection occurred. He died 1 year after the diagnosis of lung cancer was established. There have been 5 reported cases of Kartagener syndrome complicated with lung cancer, but to the best of our knowledge there have been no reports of Kartagener syndrome with mediastinal tumor.
Kartagener's syndrome Primary ciliary dyskinesia Lung cancer Mediastinal tumor
Received 平成21年8月10日
JJRS, 48(5): 375-378, 2010