ABSTRACT
BACKGROUND: Diagnosis of pulmonary artery sarcoma is difficult and frequently delayed owing to the insidious, non-specific nature of the symptoms. We present our experience with surgery for this rare disease. METHODS: Between 1995 and 1998, four patients underwent surgery for pulmonary trunk sarcomas. They presented with similar symptoms and were initially treated for thromboembolic disease of the lung. Preoperative diagnosis was established using computer tomography and echocardiography. Postoperatively, two patients received radiotherapy and one combined radio-/chemotherapy. RESULTS: One patient underwent thrombendarteriectomy of the trunk with adjuvant radiotherapy and remains tumor-free after three years and one showed complete regression of metastases after chemotherapy and remains well after more than one year. The other two patients died within two months of surgery, one due to a tumor mass in the inferior caval vein and the other because of cerebral metastases. CONCLUSIONS: According to the literature, mean survival rate is 1.5 months without resection and 10 months with resection, with survival up to four years following combined resection and adjuvant therapy. Our findings are in agreement with this.
