title: Primary sarcoma of the lung: a clinical study with long-term follow-up.
abstract: 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.
authors.affiliation: Department of Pulmonary Diseases, St. Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: J P
authors.initials: JP
authors.lastname: Janssen
authors.forename: J J
authors.initials: JJ
authors.lastname: Mulder
authors.forename: S S
authors.initials: SS
authors.lastname: Wagenaar
authors.forename: H R
authors.initials: HR
authors.lastname: Elbers
authors.forename: J M
authors.initials: JM
authors.lastname: van den Bosch
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012509:Sarcoma / Q000401:mortality / Q000473:pathology / Q000601:surgery; D015996:Survival Rate; D013908:Thoracotomy