A 41-year-old patient with no relevant morbid history presented with a one-month history of dyspnea of rapid progression associated with cough with hemoptoic expectoration and impaired general health.
On admission, an angioTAC was performed, which showed a tumoral mass, intrafamilial in left atrial septal defect to pulmonary veins.
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The echocardiogram confirmed a hyperdense mass of 43 x 38 mm occupying 75% of the left atrium.
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Approximately 95% of the tumor was surgically resected due to pulmonary veins.
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Histological examination showed the presence of a fused tumor with some oval cells, pericytes, arranged in a solid pattern, interposed by thin vascular spaces, lined by endothelial cells and flat.
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Immunohistochemistry showed positive reactivity for vimentin in proliferating cells, positive CD-34 in the endothelium of vessels and pericytes, positive factor VIII only in endothelium S-100, and negative.
These findings, together with the clear pattern of reticulin in the argentic techniques, indicated the pericitic nature of the neoplasia and its histological character was confirmed, with a mitotic index below three mitosis per 10
The patient recovered satisfactorily after surgery and was discharged.
One month later, the patient returned to the hospital with severe neck pain associated with odynophagia.
Dissemination study with PET-CP showed hypermetabolic areas in lung, skull base, liver, left tonsil, left atrium, left adrenal, pancreas tail and descending colon.
This was explained by tumor embolization to the systemic circulation.
A case was presented to the oncology committee that decided to palliative and pain management.
Two months later, the patient died of multiorgan failure.
