A 59-year-old woman with hypothyroidism began medical history in 2009, with an episode of sudden onset dyspnea and chest pain CT compatible with deep venous thrombosis (without evidence of venous thrombosis).
The patient remained on oral anticoagulant therapy with acenocoumarol and INR between 2 and 3.
In 2010, she presented a second episode manifesting as sudden dyspnea, with INR within the therapeutic range and a negative thrombophilia study.
The study of occult neoplasia was also negative and included CT of the abdomen and pelvis, upper digestive endoscopy, and gynecological evaluation.
In 2011, she suffered a third episode, with negative Doppler ultrasound again for DVT and echocardiogram, which showed high systolic pulmonary artery pressure in moderate range (53 mmHg).
INR range was adjusted to reach values between 3 and 4.
In 2012, the patient was hospitalized for a fourth episode of dyspnea suggestive of a new episode.
On this occasion, the patient reported an episode of small hemoptysis.
CT angiography showed an aneurysm of the left interlobar artery of 4.1 cm in diameter, associated with increased diameter of the pulmonary artery trunk.
She was evaluated by thoracic surgeons to define aneurysm resolution and by rheumatologists to evaluate the underlying pathology that explains the findings.
Her tests showed a normal blood count and an elevated erythrocyte sedimentation rate of 44 mm/h, with no other abnormalities.
The rheumatologic evaluation highlighted the history of a first trimester abortion with two other normal pregnancies and a daughter with systemic lupus erythematosus in treatment.
The antinuclear antibodies were positive to titres of 1; however, anti-DNA, anticardiolipin and VDRL antibodies were negative.
The eye fundus showed no relevant findings.
PET CT was proposed and could not be performed.
The evaluation by thoracic surgeons defined surgical resolution of the aneurysm due to the history of hemoptysis and the associated risk of severe hemorrhage with high mortality.
The preoperative echocardiogram showed a systolic pulmonary artery pressure of 55 mmHg, with secondary severe tricuspid regurgitation and large dilation of the right cavities, in addition to severe dilation of the left branch of the pulmonary artery.
The coronary angiography was normal and the sound of the right cavities revealed dilatation of the main pulmonary arteries, occlusion of the right middle and lower lobar branches and aneurysmal dilatation of the distal pulmonary branch echocardiogram with the same left lower pressure indicated.
The patient underwent surgery despite the changes described, due to the high mortality of a complicated aneurysm.
When dissection of the walls of the pulmonary parenchymal aneurysm was not achieved, a left lower lobectomy was performed.
A great number of thrombi were found within the aneurysm.
Upon closure of the ventricular maneuver, the patient developed an episode of fibrillation, performing resuscitation and exploratory laparotomy that showed no complications secondary to surgery.
The patient was admitted to the intensive care unit where sepsis and multiple organ failure were frequent.
Five days after surgery, the patient developed a new episode of ventricular fibrillation that did not respond to resuscitation maneuvers and died.
The analysis of the surgical specimen showed a neoplasm in fused areas, with marked cellularity and atypia, located exclusively in the arteries of the bronchovascular axes, occupying the lung layers in the same multifocal form.
Immunohistochemistry was positive only for vimentin, being negative for CD34, CD68, S100, actin, desmin and keratin AE1/AE3 and ALK-1.
We conclude grade II fibrosarcoma of the intima of the pulmonary artery and secondary branches.
The supposed intraaneurysmal thrombus corresponded to fragments of sarcoma.
