Intimal pulmonary artery sarcoma presenting as severe dyspnea and right heart insufficiency.
BACKGROUND
Pulmonary artery sarcoma is a rare tumor with a poor prognosis.


METHODS
We report the case of a 64-year-old man with an intimal pulmonary artery sarcoma presenting with severe high oxygen flow-demanding dyspnea and weight loss of 12 kg in the last 6 months. On echocardiography, right heart insufficiency, markedly elevated right ventricular pressure, a pressure gradient along the right outflow tract, and a tumor mass adherent to the wall of the truncus pulmonalis were detected. The tentative diagnosis by echocardiographic findings was pulmonary artery sarcoma. Computed tomography of the thorax and 18-fluorodeoxyglucose positron emission tomography showed an advanced local tumor manifestation. Surgical resection of the tumor to improve hemodynamics confirmed the diagnosis.


CONCLUSIONS
Pulmonary artery sarcoma should be considered as a rare differential diagnosis in patients with dyspnea due to right heart failure, particular in the case of additional weight loss, and echocardiographic examination is a useful first diagnostic approach in establishing the diagnosis.
Medizinische Klinik und Poliklinik I, Universitätsklinikum an der TU Dresden, TU Dresden, Germany. Michael.Halank@uniklinikum-dresden.de
Michael
M
Halank
Christiane
C
Jakob
Martin
M
Kolditz
Gerd
G
Hoeffken
Utz
U
Kappert
Gerhard
G
Ehninger
Matthias
M
Weise
D001707:Biopsy, Needle; D004417:Dyspnea / Q000209:etiology*; D018618:Echocardiography, Doppler, Color; D017548:Echocardiography, Transesophageal; D017809:Fatal Outcome; D006333:Heart Failure / Q000209:etiology* / Q000601:surgery; D006801:Humans; D008168:Lung / Q000473:pathology; D008207:Lymphatic Metastasis / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D011013:Pneumonectomy; D011651:Pulmonary Artery / Q000473:pathology* / Q000601:surgery; D012509:Sarcoma / Q000473:pathology* / Q000601:surgery; D013906:Thoracoscopy; D014057:Tomography, X-Ray Computed; D017539:Tunica Intima / Q000473:pathology*; D019043:Vascular Neoplasms / Q000473:pathology* / Q000601:surgery