A 7-year-old neutered female English Setter presented with syncope, anemia,
and weight loss. Clinical examination revealed a systolic murmur and echoc
ardiography demonstrated a mass on the pulmonic valve. Postmortem examinati
on confirmed the presence of a pulmonic valve mass that extended along the
pulmonary trunk and into the left pulmonary artery. Multiple pale nodules w
ere observed in the right lung. Microscopic examinations of the pulmonary a
rtery mass and the lung nodules revealed a pleomorphic population of spindl
e cells often arranged in broad bands containing strap-like nuclei and eosi
nophilic cytoplasm devoid of cross striations. The neoplastic cells express
ed vimentin and alpha-smooth muscle actin but did not express desmin, CD31,
factor VIII, or S100. The presentation, histological features, immunocytoc
hemical profiles, and behavior of this tumor were indicative of a primary p
ulmonary artery leiomyosarcoma with lung metastasis.