ObjectiveTo investigate the clinical features of primary tracheal or pulmonary malignant glomus tumor (MGT).MethodsA patient with primary tracheal MGT was reported. Wanfang, CNKI, Embase, Ovid, Cochrance and PubMed databases were searched with key words "tracheal malignant glomus tumor" and " pulmonary malignant glomus tumor” both in English and in Chinese for literature of primary tracheal or pulmonary MGT. Their clinical manifestations, imaging findings, bronchoscopic findings, pathological findings, especially immunohistochemical characteristics, diagnosis and differential diagnosis, treatment, and prognosis of primary tracheal or pulmonary MGT were summarized.ResultsThis male patient who manifested as cough, chest tightness, shortness of breath and dyspnea was diagnosed as tracheal MGT and received a treatment of radiofrequency ablation and cryotherapy under bronchoscopy. He refused to receive the chemotherapy and died after two months with a lung metastases. A total of 17 cases of primary tracheal or pulmonary MGT were retrieved in 16 articles, of which 5 were primary in the trachea and 12 were primary in the lung. Their clinical manifestations, imaging findings, and bronchoscopic findings were non-specific. The diagnosis relied on pathology, especially immunohistochemical staining. The preferred treatment of tracheal MGT may be surgical resection, the prognosis was acceptable. Treatment of lung MGT included lobectomy or airway interventional treatment and its prognosis was poor. The treatment of primary tracheal or pulmonary MGT needed early treatment. Because the curative effect of airway interventional treatment and chemotherapy was dubious, surgical plan should be first considered.ConclusionsBoth of primary tracheal and primary pulmonary MGT are rare. They usually lack specific clinical manifestations, laboratory tests, and imaging, and are easy to be misdiagnosis or miss diagnosis. The diagnosis can be confirmed by the results of biopsy for pathological analysis, especially by the immunohistochemical staining analysis. The treatment of tracheal and pulmonary MGT need further study.