Abstract
A primary pulmonary leiomyosarcoma is a very rare pulmonary malignancy that arises from smooth muscle of either the bronchial or arterial walls. Common symptoms of the tumor are cough, dyspnea, chest pain and hemoptysis. The diagnosis of a primary pulmonary sarcoma can be established only after extensive clinical and radiologic examinations have failed to identify an alternative primary source. The only effective treatment for the tumor is a complete surgical resection when feasible. The type of resection is dictated by the local anatomic extent of the tumor. We report a case of a 21-year-old male with a primary endobronchial leiomyosarcoma who presented with massive hemoptysis. A necrotic ulcerative endobronchial lesion was observed in the orifice of left lower lobe bronchus on a bronchoscopic examination. He was treated with a complete sleeve resection of the left lower lobe. Three months later, local recurrence of the tumor was noticed on the follow up bronchoscopy and a then left pneumonectomy was then performed. Fifteen months later, the patient died from empyema with a bronchopleural fistula that was associated with tumor recurrence at the stump of the pneumonectomy.
원발성 기관지내 평활근육종은 폐에 원발하는 매우 드문 악성 종양으로 통상적인 생검방법으로는 육종의 진단이 쉽지 않고 수술적 폐생검이 필요한 경우가 많으며 치료는 근치적 절제술로 완치를 기대할 수 있다. 저자들은 대량객혈을 주소로 내원한 21세 남자에서 좌폐하엽 기관지에서 발생한 원발성 기관지내 평활근육종을 진단하고 좌하엽 수상절제술을 시행하였으나 3개월 후에 국소 재발하였다. 1차 수술 후 4개월 째 좌측 전폐절제술을 시행하였으나 2차 수술 후 11개월 째 다시 국소재발과 기관지흉막루가 발생하였고 이와 관련된 농흉과 패혈증으로 사망하였던 환자를 경험하여 이를 보고하는 바이다.