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G Ital Cardiol (Rome). 2017 Nov;18(11):792-795. doi: 10.1714/2803.28366.

[A case of pulmonary artery sarcoma and review of the literature].

[Article in Italian]

Author information

S.C. Cardiologia, Presidio Ospedaliero di Ivrea-Ciriè (TO) - Dipartimento di Medicina Clinica e Sperimentale, Università degli Studi, Messina.
S.C. Anatomia Patologica, Presidio Ospedaliero di Ivrea (TO).
S.C. Cardiologia, Presidio Ospedaliero di Ivrea-Ciriè (TO).
S.S. Post-Acuzie Cardiologica, Presidio Ospedaliero di Ciriè (TO).
S.C. Radiologia, Presidio Ospedaliero di Ivrea (TO).


Pulmonary artery sarcoma (PAS) is a highly malignant tumor that originates in the pulmonary artery. This disease has a poor prognosis. Early diagnosis followed by radical surgical resection constitutes the only chance of survival for patients. However, owing to the rare and nonspecific clinical manifesta-tions and imaging findings, PAS is frequently misdiagnosed as various pulmonary thromboembolic diseases. We report the case of a 49-year-old woman who presented to our emergency department for dyspnea, hemoptysis, cough, and asthenia. A diagnosis of pulmonary thromboembolism was initially postulated. However, the rapid clinical progression of the disease, characterized by multiorgan involvement, together with the persistence of pulmonary filling defects despite appropriate anticoagulation therapy, induced to a late diagnosis of metastasized PAS. The peculiarity of our case consists of two main factors: the first is the atypical clinical presentation characterized by severe neurological impairment that finally led to patient's death; the second is the histopathological aspect of the lesion with a prevalent histiocytic cell component that is described in the literature as the less frequent pathological variant of this tumor.

[Indexed for MEDLINE]

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