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 patient s (82%) underwent operation. Radical resection is the only curative tr eatment 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 cou ld 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 follo w-up, 13.5 years). Histologic diagnosis in these patients was leiomyos arcoma in 4, malignant schwannoma in 2, and fibrosarcoma and undiffere ntiated sarcoma in I each. Median survival for all patients was 24 mon ths. Actuarial 5-year survival was 44% for all patients. Small tumor d iameter and low-grade malignancy are statistically significant favorab le 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 pulmona ry sarcoma had recurrence or metastasis.