Primary sarcoma of the lung: a clinical study with long-term follow-up.

Primary pulmonary sarcoma is an extremely rare tumor. In more than 30 years, only 22 patients with PPS were seen in our hospital; 18 patients (82%) underwent operation. Radical resection is the only curative treatment in patients with primary pulmonary sarcoma. All 4 patients (18%) who did not undergo operation died within 17 months. All 7 patients (32%) in whom no radical resection could be performed died between 10 months and 16 years after operation. Total resection of the tumor could be performed in 11 patients (50%). Of these, 7 are still alive (64%), and 1 patient died of an unrelated cause after 25 years (mean follow-up, 13.5 years). Histologic diagnosis in these patients was leiomyosarcoma in 4, malignant schwannoma in 2, and fibrosarcoma and undifferentiated sarcoma in 1 each. Median survival for all patients was 24 months. Actuarial 5-year survival was 44% for all patients. Small tumor diameter and low-grade malignancy are statistically significant favorable prognostic factors. No patient with grade 1 tumor died; the median survival was 60 months for grade 2 sarcomas, and 17 months for grade 3 sarcomas. No patient with a completely resected small primary pulmonary sarcoma had recurrence or metastasis.