P24 AN UNSUAL ENDOCARDITIS

Abstract

Abstract In February 2021 a 28 years old woman presented with effort dyspnea. She refers a history of post caesarean section sepsis treated with antibiotic therapy. At TTE and following TEE: thickening of the interatrial septum, protruding neoformation in the left atrium as well as mitral valve steno–insufficency related to infiltration of the neoformation. The patient was then referred for cardiac surgery in Vicenza. The resection of the neoformation infiltrating the left atrium, interatrial septum, leaflet, annulus and papillary muscles of the mitral valve was performed (minithoracotomy). Finally valve replacement with Hanckock bioprosthesis no. 27 was performed. Extemporaneous histological examination showed a possible intimal sarcoma, which was subsequently confirmed (G3, IV stadium, MDM2+). At CT angiography evidence of vascular involvement (VCI and pulmonary vein) and possible secondary sites. The patient was referred to the regional reference centre (C.R.O. Aviano) where she was treated with six cycles of chemotherapy with EpiADM and Ifofosfamide, resulting in excellent remission of the tumour. At control PET/CT with FDG (01/22) substantial absence of disease. Intimal sarcoma (ISA) is an exceedingly rare undifferentiated sarcoma that arises in the pulmonary artery and less frequently in the aorta or its branches. ISAs behave highly aggressive with a mean patients’ survival ranging from 5 to 18 months. Patients with ISA are mostly of middle age at diagnosis and typically present with non–specific symptoms, which sometimes masquerades as thromboembolic disease. Thus, patients are often diagnosed in an advanced disease stage. Furthermore, ISAs are often reported to be resistant to conventional chemotherapy.