P1700 Recurrent inflammatory myofibroblastic tumor of the right ventricle and pulmonary artery

Abstract

Introduction Inflammatory myofibroblastic tumor (IMT), also known as plasma granuloma, is a very rare tumor with varying biological behavior, from completely benign to malignant. IMT can occur in virtually every organ, but cardiac origin is extremely rare. IMT occurs mainly in children and young people. The mainstay of treatment is complete surgical resection of the tumor. We present a case of 28 year old white woman with no contributory medical and family history, who was admitted to hospital because of dyspnea and cough. The transthoracic echocardiography (TTE) showed elevated pulmonary artery systolic pressure up to 61 mm Hg and mild dilation of right chambers. Further assessment by computed tomography showed thrombosis of the main pulmonary artery (PA), narrowing its lumen by 90%, with spread to the lobar and segmental arteries of the left lung - signs of massive pulmonary embolism. During thromboendarterectomy revealed a mass, about 4,1 x 3,1 x 1,8 cm in size, with thrombotic overlays, locating at the right ventricular outflow tract (RVOT) and the pulmonary trunk. Histological examination and Immunohistochemistry studies was consistent with IMT with an intermediate potential of malignancy. A year after the operation, the patient became pregnant. Routine TTE showed low density mobile mass narrowing the pulmonary trunk. The transvalvular blood flow velocity was 2.4 m/s. Cardiac magnetic resonance (CMR) was consistent with echo findings. The pregnancy has been preserved, and after 36 weeks it was decided to perform the birth of a girl by means of a Cesarian section. After 2 months, due to an increase in the size of the tumor and the dyspnea, a reoperation was performed to remove the tumor lining the RVOT and intimately connected with the PA valve, excision of the PA valve. A bioprosthesis was installed. The tumor had an identical morphological picture. Conclusion This case report describes a rare recurrent cardiac inflammatory myofibroblastic tumor involving RVOT and PA. The prognosis after surgical treatment of IMT is usually favorable, but it is known that some tumors recur, and rarely they can even metastasize. The long-term outcome is not well established due to the rarity of these tumors. Abstract P1700 Figure.