Abstract
INTRODUCTION: Primary leiomyosarcomas (LMS) of the mediastinum are extremely rare malignant mesenchymal tumors developing from soft tissues or great vessels. We present a case of a primary leiomyosarcoma of the middle mediastinum in which long-term survival was achieved. CASE REPORT: A 77-year-old man presented to us for examination with an extrapleural sign in his upper mediastinum on chest X-ray. Computed tomography (CT) revealed a well-circumscribed mass in the middle mediastinum. Thoracoscopic resection of the mediastinal tumor and immunohistological findings, which were positive for smooth muscle actin (SMA), HHF-35, vimentin, and desmin confirmed primary leiomyosarcoma. It recurred twice with solid right pulmonary metastases, which were resected. He was followed-up for a total of 9 years and 6 months from the first surgery with no signs of recurrence after his last surgery. CONCLUSION: Surgical resection of both the primary tumor and pulmonary metastases remains the mainstay of treatment of primary leiomyosarcomas.