Pulmonary artery sarcoma: a rare cause of dyspnoea.
A 72-year-old woman with no significant medical history presented to the emergency room for severe dyspnoea. The initial clinical diagnosis was acute pulmonary embolism. Heparin infusion was initiated while awaiting a computed tomographic scan but the patient's condition deteriorated dramatically and stat echocardiogram showed tamponade. Post-evacuation echo showed a dilated right ventricle with pulmonary hypertension and obstruction of the right pulmonary artery by a homogeneous mass attached to the pulmonary artery, suggesting a tumour rather than a thrombus. Computed tomographic scan confirmed the presence of an obstructive mass with almost no perfusion of the right lung. The patient was referred to cardiac surgery and the mass was removed, with anatomo-pathological diagnosis of a typical pulmonary artery sarcoma. Unfortunately, the patient died a few days after surgery. Primary pulmonary artery sarcoma is a rare tumour that arises in the central pulmonary arteries. Clinical presentation is often attributed to other causes of pulmonary hypertension, like pulmonary embolism. Magnetic resonance imaging could help to distinguish a soft tissue mass from a thrombus but definitive diagnosis is almost always made at surgery or autopsy since patients usually present in acute and unstable situations. Prognosis is poor, from several months to a few years, and depends on how early the diagnosis is made, the presence of recurrence or metastasis after surgical resection, and the use of adjuvant therapy like radiation and chemotherapy.
Service de Cardiologie, Hôpital Lariboisière, APHP, Paré, Paris, France.
Najia
N
Chaachoui
William
W
Haik
François
F
Tournoux
D000368:Aged; D003937:Diagnosis, Differential; D004417:Dyspnea / Q000175:diagnosis / Q000209:etiology*; D015150:Echocardiography, Doppler / Q000379:methods; D004636:Emergency Service, Hospital; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D011651:Pulmonary Artery; D011655:Pulmonary Embolism / Q000175:diagnosis / Q000000981:diagnostic imaging*; D035583:Rare Diseases; D018570:Risk Assessment; D012509:Sarcoma / Q000150:complications* / Q000000981:diagnostic imaging* / Q000601:surgery; D019043:Vascular Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging* / Q000601:surgery; D014656:Vascular Surgical Procedures / Q000379:methods