Abstract
Pulmonary artery intimal sarcoma (PAIS) is an extremly rare and agressive tumor. The prognosis is very poor, with median overall survival between 11 and 18 months. We report a case of a 38 year old man who presented to our oncology department with diagnosis of PAIS confirmed by histological examination after surgical resection and bioprosthetic pulmonary valve replacement. Six months after adjuvant chemotherapy, the patient presented a recurrence of his disease that requires other lines of chemotherapy with prolonged survival.
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