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Sarcoma
Volume 2017, Article ID 7941432, 6 pages
https://doi.org/10.1155/2017/7941432
Research Article

Multimodal Approach of Pulmonary Artery Intimal Sarcoma: A Single-Institution Experience

1Division of Medical Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
2Cardiothoracic Surgery Department, Fondazione IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy
3Service of Biometry & Statistics, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
4Unit of Pulmonary Rehabilitation, ICS Maugeri Istituto Scientifico di Montescano, Pavia, Italy
5Unit of Pathology, Fondazione IRCCS Policlinico San Matteo and Pavia University School of Medicine, Pavia, Italy
6Division of Radiology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

Correspondence should be addressed to S. Secondino; ti.vp.oettams@onidnoces.s

Received 18 April 2017; Accepted 5 July 2017; Published 20 August 2017

Academic Editor: Akira Kawai

Copyright © 2017 S. Secondino et al. 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.

Abstract

Introduction. Pulmonary artery sarcoma (PAS) is a rare tumor, whose therapeutic approach is mainly based on surgery, either pneumonectomy or pulmonary endarterectomy (PEA). The prognosis reported in published series is very poor, with survival of 1.5 months without any kind of treatment. Patients and Methods. From January 2010 to January 2016, 1027 patients were referred to our hospital for symptoms of acute or chronic pulmonary thromboembolic disease. Twelve patients having a confirmed diagnosis of PAS underwent PEA. Median age was 64.5 years. Most patients had a long history of symptoms, having a median time of 7.5 months from onset of symptoms to surgery. Results. Following PEA and cardiopulmonary rehabilitation, 10 patients received conventional chemotherapy with doxorubicin and ifosfamide, starting at a median of 42 days from surgery. Four patients also received radiotherapy. Four patients have died due to disease progression, while 7 are still alive, with 5 being disease-free at 4–55+ months from diagnosis. Conclusions. In patients with PAS, a multimodal approach including PEA, CT, and RT is feasible but it should be evaluated individually, according to the tumor extension and the patient’s clinical condition. Apart from improving quality of life mainly by reducing or delaying symptoms due to PH, it may improve life expectancy.