Primary bronchopulmonary leiomyosarcoma of the left main bronchus in a child presenting with wheezing and atelectasis of the left lung.

摘要

Primary lung tumors are rare in children, and primary bronchopulmonary leiomyosarcomas are very rare in children, with only 10 cases reported in the English-language literature. We report on the eleventh case of primary bronchopulmonary leiomyosarcoma in a child, and it is the first case in Taiwan, and also in Asia. In addition, this is the second case of this tumor arising in the left main bronchus. Before the present case, there were only 2 cases of this tumor in the main bronchus: one was in the right main-stem bronchus, and the other in the left-stem main bronchus. Ours is the second case showing pleural effusion. Chest roentgenogram and bronchoscopy are helpful in the diagnosis of primary bronchopulmonary tumors, but a definitive diagnosis can only be made by biopsy. The tumor is potentially curable if total excision is possible; irradiation has little therapeutic value, and early excision offers the only hope of cure. The child is alive and well 19 months after pneumonectomy, without having received radiotherapy or chemotherapy. Copyright 2002 Wiley-Liss, Inc.