Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma

OBJECTIVES The study aimed to determine the optimal surgical procedure to treat pulmonary artery sarcomas responsible for pulmonary hypertension. METHODS Between 1997 and 2010, 31 patients were treated surgically for pulmonary artery sarcomas. Sixteen patients were male; the mean age was 56 years (r...

Full description

Saved in:
Bibliographic Details
Published in:European journal of cardio-thoracic surgery 2013-04, Vol.43 (4), p.787-793
Main Authors: Mussot, Sacha, Ghigna, Maria-Rosa, Mercier, Olaf, Fabre, Dominique, Fadel, Elie, Le Cesne, Axel, Simonneau, Gerald, Dartevelle, Philippe
Format: Article
Language:English
Subjects:
Adult
Aged
Angioscopy - methods
Endarterectomy - methods
Female
Humans
Hypertension, Pulmonary - physiopathology
Kaplan-Meier Estimate
Male
Middle Aged
Multimodal Imaging
Positron-Emission Tomography
Pulmonary Artery - diagnostic imaging
Pulmonary Artery - pathology
Pulmonary Artery - surgery
Retrospective Studies
Sarcoma - diagnosis
Sarcoma - surgery
Tomography, X-Ray Computed
Vascular Neoplasms - diagnosis
Vascular Neoplasms - surgery
Video-Assisted Surgery - methods
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:OBJECTIVES The study aimed to determine the optimal surgical procedure to treat pulmonary artery sarcomas responsible for pulmonary hypertension. METHODS Between 1997 and 2010, 31 patients were treated surgically for pulmonary artery sarcomas. Sixteen patients were male; the mean age was 56 years (range, 26-78 years). Common symptoms were characteristic of acute or chronic pulmonary thromboembolic disease. Also, 21 patients experienced mild to severe pulmonary hypertension, with a mean total peripheral resistance of 473 dyn s cm−5. Clinical presentation and preoperative work-up confirmed the suspicion of pulmonary artery sarcoma in 18 patients. The required surgical procedures included the following: pulmonary endarterectomy in 25 patients (combined with a right pneumonectomy in five and with a replacement of the main pulmonary artery by a homograft reconstruction in one), pneumonectomy only in five (three right and two left), with the use of cardiopulmonary bypass in three cases. In one patient, the right pulmonary artery only was replaced on cardiopulmonary bypass. RESULTS Final pathology showed 26 high-grade and five intermediate-grade sarcomas. The 30-day mortality was 13% (four patients). Repeat pulmonary resection was required in two patients due to recurrent disease. Moreover, 18 patients received adjuvant therapy. Mean follow-up was 19 months (range, 1-99 months); of the 11 patients alive at follow-up, four were noted to have recurrent disease. The 1-, 3- and 5- year survival was 63, 29 and 22%, respectively. CONCLUSIONS The prognosis of this very infrequent disease remains poor. Bilateral pulmonary endarterectomy may yield significant survival rates because it provides completeness of resection without sacrificing the pulmonary vascular bed.
ISSN:1010-7940
1873-734X
DOI:10.1093/ejcts/ezs387