Background: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms.
Design: Retrospective review of medical records.
Purpose: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis.
Patients: The study group consisted of 12 patients, with a mean age of 53 years.
Results: The main symptoms were chest pain, and cough. Imaging findings consisted of: eight single peripheral opacities, three single parahilar opacities, and one lobar actelectasis. The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma. Thoracic surgery done in nine cases consisted of six lobectomies with further parietal resection in two cases, and three pneumonectomies. Four patients received chemotherapy and two patients had radiation therapy postoperatively. Follow up available on 12 patients ranged from 3 to 144 (mean 42) months. Long term survival up to 3 years was observed in five patients. Median overall survival was 48 months. Overall 5-year survival rate was 38%.
Conclusions: Primary sarcomas of the lung are a rare and aggressive malignancy. Treatment and prognosis do not differ from other soft tissue sarcomas.