[Surgical management of pulmonary artery sarcoma].

BACKGROUND AND OBJECTIVES: Pulmonary artery sarcoma is a rare neoplasm and possibly unnoticed cause of pulmonary hypertension. The presentation is one of central pulmonary artery obstruction and progressive right-heart failure. In most cases, the diagnosis of malignancy is confirmed post mortem. We report the outcome of eight patients with primary pulmonary artery sarcomas.

METHODS: Eight patients (four female, four male, mean age 48,2 years, preop. NYHA functional class III/IV: n = 5/3) were referred for further evaluation of pulmonary hypertension. Malignancy was suspected in six of these patients by means of computed tomography (CT) and magnetic resonance tomography (MRT). In two patients diagnosis was established during pulmonary thromboendarterectomy based on histological examination of frozen sections. Operative procedures consisted of gross tumor resection with prosthetic replacement (n = 3) or reconstruction (n = 5) of central parts of the pulmonary vessels. Additional pneumonectomy was necessary in two patients, resection of metastases in one patient. Seven patients received adjuvant radio- and/or chemotherapy.

RESULTS: There were no postoperative deaths. 3 months after surgery, all patients demonstrated improvement in hemodynamics and exercise tolerance. Four patients died 7, 9, 18 and 19 months after surgery, respectively. Two patients are alive 3 and 39 months after primary surgery with evidence of pulmonary metastases. Two patients are alive in complete remission 25 and 65 months postoperatively.

CONCLUSIONS: In patients with primary pulmonary artery sarcoma, emphasis must be placed on early identification which can be achieved by CT and MRT. Radical surgical resection currently offers the best chance for survival. Adjuvant therapy might bring additional benefit.