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S. Secondino [1] and V. Grazioli [2] and F. Valentino [1] and M. Pin [2] and A. Pagani [1] and A. Sciortino [2] and C. Klersy [3] and M. G. Callegari [4] and P. Morbini [5] and R. Dore [6] and M. Paulli [5] and P. Pedrazzoli [1] and A. M. D’armini [2]

Academic Editor: Akira Kawai

1, Division of Medical Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, sanmatteo.org

2, Cardiothoracic Surgery Department, Fondazione IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy, unipv.eu

3, Service of Biometry & Statistics, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, sanmatteo.org

4, Unit of Pulmonary Rehabilitation, ICS Maugeri Istituto Scientifico di Montescano, Pavia, Italy, fsm.it

5, Unit of Pathology, Fondazione IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy, unipv.eu

6, Division of Radiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, sanmatteo.org

Received Apr 18, 2017; Accepted Jul 5, 2017

This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

1. Introduction

Pulmonary artery sarcoma (PAS) was first described in 1923 [1]. Since that time, about 300 PAS have been reported in the English-language literature, as case reports or small series. Primary PAS is the most frequent sarcoma of the great arteries [2], in most cases arising in the area derived from the embryologic bulbus cordis, which leads to PAS predominantly occurring in the main pulmonary artery [3] which is involved in 80% of cases: the left pulmonary artery in 58%, the right pulmonary artery in 57%, both arteries in 37%, the pulmonary valve in 29%, and the right ventricle in 8%.

Primary PAS are classified by location as either luminal or mural in origin and then further subclassified histologically. Luminal PAS are thought to derive from pluripotential mesenchymal cells of the intima, while mural sarcomas, less frequent than luminal sarcomas, can be difficult to distinguish from sarcomas of the lung parenchyma due to their growing; they are more likely to appear as mass-like lesions [4]. PAS typically affect middle-aged people, favoring women slightly. Common symptoms at time of presentation often mirror those of pulmonary embolism, including progressive dyspnea, cough, and...
 
 
 
 
 
 
 

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