The prognosis of primary angiosarcoma of the pulmonary system is generally poor, irrespective of whether the location is in the pulmonary parenchyma or the main airway. Angiosarcoma of the pulmonary parenchyma is invariably fatal within months because metastases are usually present at the time of detection, and chemotherapy thus far has not proved effective. The clinical course of solitary angiosarcoma of the main airway is also dismal in previous reports. 2,4 However, mortality of these patients was mainly caused by massive and intractable hemoptysis of the small resectable tumor rather than by the invasiveness of the disease. From the experience of successful treatment of this patient, angiosarcoma should be considered in the differential diagnosis of a primary tracheal tumor. Furthermore, prompt surgical intervention with adequate resection margins is recommended in the management of these patients when the disease is localized at detection.
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