Surgical treatment of primary pulmonary artery sarcoma.
OBJECTIVE
Primary pulmonary artery sarcoma is a severe and underdiagnosed disease, with the clinical and surgical approach not clearly established. Only a few individual case reports or small series on this topic have been published. The aim of the present study was to report our surgical experience in this field.


METHODS
From March 2004 to December 2012, 13 patients underwent surgery for pulmonary artery sarcoma at our institution. In 7 patients, the sarcoma was unilateral (53.8%), and in 6 (46.2%), the tumor had already extended to both lungs. The surgical strategy evolved over the years, but the 2 techniques used were always the same: pneumonectomy in 5 patients and pulmonary endarterectomy in 8.


RESULTS
Two patients died in-hospital, both in the pneumonectomy group. The median length of the intensive care unit and hospital stay was 1 day (range, 1-10) and 14 days (range, 11-17) for the pneumonectomy group and 6 days (range, 3-23) and 19 days (range, 10-32) fort the pulmonary endarterectomy group, respectively. The median survival was 26.8 months after pneumonectomy and 6.6 months after pulmonary endarterectomy.


CONCLUSIONS
Primary pulmonary artery sarcoma has a poor prognosis. The surgical strategy at our institution included pneumonectomy, for possible radical resection, and palliative endarterectomy, to reduce symptoms and increase the life expectancy. The correct surgical approach must be evaluated individually, according to the tumor presentation, the presence of pulmonary hypertension, and the patient's clinical condition.
Division of Cardiothoracic Surgery, IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy.
Valentina
V
Grazioli
Division of Cardiothoracic Surgery, IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy.
Nicola
N
Vistarini
Division of Cardiothoracic Surgery, IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy.
Marco
M
Morsolini
Service of Biometrics and Statistics, IRCCS Policlinico San Matteo, Pavia, Italy.
Catherine
C
Klersy
Division of Cardiothoracic Surgery, IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy.
Giulio
G
Orlandoni
Division of Radiology, IRCCS Policlinico San Matteo, Pavia, Italy.
Roberto
R
Dore
Division of Cardiothoracic Surgery, IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy. Electronic address: darmini@smatteo.pv.it.
Andrea Maria
AM
D'Armini
D000328:Adult; D000368:Aged; D004691:Endarterectomy / Q000009:adverse effects / Q000401:mortality; D005260:Female; D017052:Hospital Mortality; D006801:Humans; D007362:Intensive Care Units; D007902:Length of Stay; D008168:Lung / Q000473:pathology / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D010166:Palliative Care; D011013:Pneumonectomy / Q000009:adverse effects / Q000401:mortality; D011651:Pulmonary Artery / Q000473:pathology / Q000601:surgery*; D012509:Sarcoma / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome; D019043:Vascular Neoplasms / Q000401:mortality / Q000473:pathology / Q000601:surgery*