Pulmonary artery intimal sarcomas tend to be presented with symptoms o
f pulmonary thromboembolism and grow regionally, with little capacity
to metastasize. They probably originate from subendothelial cells, tha
t become myofibroblasts. Knowledge of it is important to establish a p
resurgery diagnosis, with the possibility of a total resection, the on
ly useful treatment until now. We report a case of a pulmonary artery
primary sarcoma, in a 73 year old woman, admitted with hemopthisis and
pleuritic chest pain, who died ten days after. Autopsy revealed an in
traluminal mass at the pulmonary artery trunk, without regional nor di
stance involvement. Microscopic study showed a pleomorphic tumor with
spindle and epithelioid cells, positive for actin, desmin and vimentin
. All these data support the diagnosis of primary intimal sarcoma of t
he pulmonary artery. We want to emphasize the myogenic differentiation
of the tumor, uncommon in previously reported cases.