Surgical treatment of pulmonary artery sarcoma

Objective: Pulmonary artery sarcomas are rare and usually fatal tumors. The diagnosis is difficult and delayed in most cases. Newer imaging techniques could allow early diagnosis in patients with symptoms of pulmonary vascula r obstruction. Surgical resection improves clinical symptoms and offers the only chance of cure. We report the case histories of 7 patients with prima ry pulmonary artery sarcomas treated by surgical resection with or without adjuvant therapy. Methods: Seven patients (3 women and 4 men; mean age, 52.3 years; preoperat ive New York Heart Association functional class III/IV, n = 5/2) underwent operations. Malignancy was preoperatively suspected in 5 patients, and 2 pa tients had a presumptive diagnosis of chronic pulmonary embolism. Tumor res ection with partial or total prosthetic replacement (n = 2), reconstruction (n = 5), or both, of central parts of the pulmonary arteries was performed in 6 patients. Thromboendarterectomy was necessary in 3 patients, and pneu monectomy was necessary in 2 patients. Six patients received adjuvant thera py. Results: There was no perioperative mortality. All patients had a substanti al improvement in exercise tolerance and hemodynamics 3 months after their operations. Four patients died 7, 9, 18, and 19 months after their operatio ns because of recurrent tumor or pulmonary metastases. Two patients are ali ve 21 and 35 months after primary surgical repair, with pulmonary metastase s detected by computed tomographic scans. One patient is alive 62 months af ter resection without clinical or radiologic signs of tumor recurrence or m etastasis. Conclusions: Early diagnosis of primary pulmonary artery sarcomas can be im proved by computed tomography and magnetic resonance scanning. Radical surg ical resection probably presents the only chance for cure. The role of neoa djuvant or adjuvant treatment modalities has to be defined. Pulmonary arter y sarcoma need not necessarily be a fatal diagnosis.